GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice Article Swipe
Martijn J. C. van der Lienden
,
Jan Aten
,
André R. A. Marques
,
Ingeborg S. E. Waas
,
Per Larsen
,
Nike Claessen
,
Nicole N. van der Wel
,
Roelof Ottenhoff
,
Marco van Eijk
,
Johannes M. F. G. Aerts
·
YOU?
·
· 2021
· Open Access
·
· DOI: https://doi.org/10.3390/ijms22052532
YOU?
·
· 2021
· Open Access
·
· DOI: https://doi.org/10.3390/ijms22052532
The lysosomal storage disease Niemann–Pick type C (NPC) is caused by impaired cholesterol efflux from lysosomes, which is accompanied by secondary lysosomal accumulation of sphingomyelin and glucosylceramide (GlcCer). Similar to Gaucher disease (GD), patients deficient in glucocerebrosidase (GCase) degrading GlcCer, NPC patients show an elevated glucosylsphingosine and glucosylated cholesterol. In livers of mice lacking the lysosomal cholesterol efflux transporter NPC1, we investigated the expression of established biomarkers of lipid-laden macrophages of GD patients, their GCase status, and content on the cytosol facing glucosylceramidase GBA2 and lysosomal integral membrane protein type B (LIMP2), a transporter of newly formed GCase to lysosomes. Livers of 80-week-old Npc1−/− mice showed a partially reduced GCase protein and enzymatic activity. In contrast, GBA2 levels tended to be reciprocally increased with the GCase deficiency. In Npc1−/− liver, increased expression of lysosomal enzymes (cathepsin D, acid ceramidase) was observed as well as increased markers of lipid-stressed macrophages (GPNMB and galectin-3). Immunohistochemistry showed that the latter markers are expressed by lipid laden Kupffer cells. Earlier reported increase of LIMP2 in Npc1−/− liver was confirmed. Unexpectedly, immunohistochemistry showed that LIMP2 is particularly overexpressed in the hepatocytes of the Npc1−/− liver. LIMP2 in these hepatocytes seems not to only localize to (endo)lysosomes. The recent recognition that LIMP2 harbors a cholesterol channel prompts the speculation that LIMP2 in Npc1−/− hepatocytes might mediate export of cholesterol into the bile and thus protects the hepatocytes.
Related Topics
Concepts
NPC1
Acid sphingomyelinase
Niemann–Pick disease, type C
Niemann–Pick disease
Sphingomyelin
Cholesterol
Sphingolipid
Endocrinology
Ceramide
Biology
Ganglioside
Internal medicine
Sphingomyelin phosphodiesterase
Hepatocyte
Chemistry
Biochemistry
Endosome
Medicine
Intracellular
In vitro
Apoptosis
Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3390/ijms22052532
- https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655
- OA Status
- gold
- Cited By
- 35
- References
- 41
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W3134294420
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
-
https://openalex.org/W3134294420Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.3390/ijms22052532Digital Object Identifier
- Title
-
GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C MiceWork title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2021Year of publication
- Publication date
-
2021-03-03Full publication date if available
- Authors
-
Martijn J. C. van der Lienden, Jan Aten, André R. A. Marques, Ingeborg S. E. Waas, Per Larsen, Nike Claessen, Nicole N. van der Wel, Roelof Ottenhoff, Marco van Eijk, Johannes M. F. G. AertsList of authors in order
- Landing page
-
https://doi.org/10.3390/ijms22052532Publisher landing page
- PDF URL
-
https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655Direct link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
-
https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655Direct OA link when available
- Concepts
-
NPC1, Acid sphingomyelinase, Niemann–Pick disease, type C, Niemann–Pick disease, Sphingomyelin, Cholesterol, Sphingolipid, Endocrinology, Ceramide, Biology, Ganglioside, Internal medicine, Sphingomyelin phosphodiesterase, Hepatocyte, Chemistry, Biochemistry, Endosome, Medicine, Intracellular, In vitro, ApoptosisTop concepts (fields/topics) attached by OpenAlex
- Cited by
-
35Total citation count in OpenAlex
- Citations by year (recent)
-
2025: 1, 2024: 1, 2023: 2, 2022: 2, 2021: 29Per-year citation counts (last 5 years)
- References (count)
-
41Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
Full payload
| id | https://openalex.org/W3134294420 |
|---|---|
| doi | https://doi.org/10.3390/ijms22052532 |
| ids.doi | https://doi.org/10.3390/ijms22052532 |
| ids.mag | 3134294420 |
| ids.pmid | https://pubmed.ncbi.nlm.nih.gov/33802460 |
| ids.openalex | https://openalex.org/W3134294420 |
| fwci | 5.43146821 |
| mesh[0].qualifier_ui | |
| mesh[0].descriptor_ui | D000818 |
| mesh[0].is_major_topic | False |
| mesh[0].qualifier_name | |
| mesh[0].descriptor_name | Animals |
| mesh[1].qualifier_ui | Q000502 |
| mesh[1].descriptor_ui | D001692 |
| mesh[1].is_major_topic | False |
| mesh[1].qualifier_name | physiology |
| mesh[1].descriptor_name | Biological Transport |
| mesh[2].qualifier_ui | Q000378 |
| mesh[2].descriptor_ui | D002402 |
| mesh[2].is_major_topic | False |
| mesh[2].qualifier_name | metabolism |
| mesh[2].descriptor_name | Cathepsin D |
| mesh[3].qualifier_ui | |
| mesh[3].descriptor_ui | D002460 |
| mesh[3].is_major_topic | False |
| mesh[3].qualifier_name | |
| mesh[3].descriptor_name | Cell Line |
| mesh[4].qualifier_ui | |
| mesh[4].descriptor_ui | D045744 |
| mesh[4].is_major_topic | False |
| mesh[4].qualifier_name | |
| mesh[4].descriptor_name | Cell Line, Tumor |
| mesh[5].qualifier_ui | Q000378 |
| mesh[5].descriptor_ui | D005776 |
| mesh[5].is_major_topic | False |
| mesh[5].qualifier_name | metabolism |
| mesh[5].descriptor_name | Gaucher Disease |
| mesh[6].qualifier_ui | Q000378 |
| mesh[6].descriptor_ui | D005962 |
| mesh[6].is_major_topic | False |
| mesh[6].qualifier_name | metabolism |
| mesh[6].descriptor_name | Glucosylceramidase |
| mesh[7].qualifier_ui | Q000378 |
| mesh[7].descriptor_ui | D005963 |
| mesh[7].is_major_topic | False |
| mesh[7].qualifier_name | metabolism |
| mesh[7].descriptor_name | Glucosylceramides |
| mesh[8].qualifier_ui | |
| mesh[8].descriptor_ui | D056945 |
| mesh[8].is_major_topic | False |
| mesh[8].qualifier_name | |
| mesh[8].descriptor_name | Hep G2 Cells |
| mesh[9].qualifier_ui | Q000378 |
| mesh[9].descriptor_ui | D022781 |
| mesh[9].is_major_topic | False |
| mesh[9].qualifier_name | metabolism |
| mesh[9].descriptor_name | Hepatocytes |
| mesh[10].qualifier_ui | |
| mesh[10].descriptor_ui | D006801 |
| mesh[10].is_major_topic | False |
| mesh[10].qualifier_name | |
| mesh[10].descriptor_name | Humans |
| mesh[11].qualifier_ui | Q000378 |
| mesh[11].descriptor_ui | D008099 |
| mesh[11].is_major_topic | False |
| mesh[11].qualifier_name | metabolism |
| mesh[11].descriptor_name | Liver |
| mesh[12].qualifier_ui | Q000378 |
| mesh[12].descriptor_ui | D051907 |
| mesh[12].is_major_topic | False |
| mesh[12].qualifier_name | metabolism |
| mesh[12].descriptor_name | Lysosomal Membrane Proteins |
| mesh[13].qualifier_ui | Q000378 |
| mesh[13].descriptor_ui | D008247 |
| mesh[13].is_major_topic | False |
| mesh[13].qualifier_name | metabolism |
| mesh[13].descriptor_name | Lysosomes |
| mesh[14].qualifier_ui | Q000378 |
| mesh[14].descriptor_ui | D008264 |
| mesh[14].is_major_topic | False |
| mesh[14].qualifier_name | metabolism |
| mesh[14].descriptor_name | Macrophages |
| mesh[15].qualifier_ui | |
| mesh[15].descriptor_ui | D051379 |
| mesh[15].is_major_topic | False |
| mesh[15].qualifier_name | |
| mesh[15].descriptor_name | Mice |
| mesh[16].qualifier_ui | |
| mesh[16].descriptor_ui | D008807 |
| mesh[16].is_major_topic | False |
| mesh[16].qualifier_name | |
| mesh[16].descriptor_name | Mice, Inbred BALB C |
| mesh[17].qualifier_ui | Q000378 |
| mesh[17].descriptor_ui | D052556 |
| mesh[17].is_major_topic | False |
| mesh[17].qualifier_name | metabolism |
| mesh[17].descriptor_name | Niemann-Pick Disease, Type C |
| mesh[18].qualifier_ui | |
| mesh[18].descriptor_ui | D000067996 |
| mesh[18].is_major_topic | False |
| mesh[18].qualifier_name | |
| mesh[18].descriptor_name | RAW 264.7 Cells |
| mesh[19].qualifier_ui | Q000378 |
| mesh[19].descriptor_ui | D051116 |
| mesh[19].is_major_topic | False |
| mesh[19].qualifier_name | metabolism |
| mesh[19].descriptor_name | Receptors, Scavenger |
| mesh[20].qualifier_ui | Q000378 |
| mesh[20].descriptor_ui | D013109 |
| mesh[20].is_major_topic | False |
| mesh[20].qualifier_name | metabolism |
| mesh[20].descriptor_name | Sphingomyelins |
| mesh[21].qualifier_ui | |
| mesh[21].descriptor_ui | D000818 |
| mesh[21].is_major_topic | False |
| mesh[21].qualifier_name | |
| mesh[21].descriptor_name | Animals |
| mesh[22].qualifier_ui | Q000502 |
| mesh[22].descriptor_ui | D001692 |
| mesh[22].is_major_topic | False |
| mesh[22].qualifier_name | physiology |
| mesh[22].descriptor_name | Biological Transport |
| mesh[23].qualifier_ui | Q000378 |
| mesh[23].descriptor_ui | D002402 |
| mesh[23].is_major_topic | False |
| mesh[23].qualifier_name | metabolism |
| mesh[23].descriptor_name | Cathepsin D |
| mesh[24].qualifier_ui | |
| mesh[24].descriptor_ui | D002460 |
| mesh[24].is_major_topic | False |
| mesh[24].qualifier_name | |
| mesh[24].descriptor_name | Cell Line |
| mesh[25].qualifier_ui | |
| mesh[25].descriptor_ui | D045744 |
| mesh[25].is_major_topic | False |
| mesh[25].qualifier_name | |
| mesh[25].descriptor_name | Cell Line, Tumor |
| mesh[26].qualifier_ui | Q000378 |
| mesh[26].descriptor_ui | D005776 |
| mesh[26].is_major_topic | False |
| mesh[26].qualifier_name | metabolism |
| mesh[26].descriptor_name | Gaucher Disease |
| mesh[27].qualifier_ui | Q000378 |
| mesh[27].descriptor_ui | D005962 |
| mesh[27].is_major_topic | False |
| mesh[27].qualifier_name | metabolism |
| mesh[27].descriptor_name | Glucosylceramidase |
| mesh[28].qualifier_ui | Q000378 |
| mesh[28].descriptor_ui | D005963 |
| mesh[28].is_major_topic | False |
| mesh[28].qualifier_name | metabolism |
| mesh[28].descriptor_name | Glucosylceramides |
| mesh[29].qualifier_ui | |
| mesh[29].descriptor_ui | D056945 |
| mesh[29].is_major_topic | False |
| mesh[29].qualifier_name | |
| mesh[29].descriptor_name | Hep G2 Cells |
| mesh[30].qualifier_ui | Q000378 |
| mesh[30].descriptor_ui | D022781 |
| mesh[30].is_major_topic | False |
| mesh[30].qualifier_name | metabolism |
| mesh[30].descriptor_name | Hepatocytes |
| mesh[31].qualifier_ui | |
| mesh[31].descriptor_ui | D006801 |
| mesh[31].is_major_topic | False |
| mesh[31].qualifier_name | |
| mesh[31].descriptor_name | Humans |
| mesh[32].qualifier_ui | Q000378 |
| mesh[32].descriptor_ui | D008099 |
| mesh[32].is_major_topic | False |
| mesh[32].qualifier_name | metabolism |
| mesh[32].descriptor_name | Liver |
| mesh[33].qualifier_ui | Q000378 |
| mesh[33].descriptor_ui | D051907 |
| mesh[33].is_major_topic | False |
| mesh[33].qualifier_name | metabolism |
| mesh[33].descriptor_name | Lysosomal Membrane Proteins |
| mesh[34].qualifier_ui | Q000378 |
| mesh[34].descriptor_ui | D008247 |
| mesh[34].is_major_topic | False |
| mesh[34].qualifier_name | metabolism |
| mesh[34].descriptor_name | Lysosomes |
| mesh[35].qualifier_ui | Q000378 |
| mesh[35].descriptor_ui | D008264 |
| mesh[35].is_major_topic | False |
| mesh[35].qualifier_name | metabolism |
| mesh[35].descriptor_name | Macrophages |
| mesh[36].qualifier_ui | |
| mesh[36].descriptor_ui | D051379 |
| mesh[36].is_major_topic | False |
| mesh[36].qualifier_name | |
| mesh[36].descriptor_name | Mice |
| mesh[37].qualifier_ui | |
| mesh[37].descriptor_ui | D008807 |
| mesh[37].is_major_topic | False |
| mesh[37].qualifier_name | |
| mesh[37].descriptor_name | Mice, Inbred BALB C |
| mesh[38].qualifier_ui | Q000378 |
| mesh[38].descriptor_ui | D052556 |
| mesh[38].is_major_topic | False |
| mesh[38].qualifier_name | metabolism |
| mesh[38].descriptor_name | Niemann-Pick Disease, Type C |
| mesh[39].qualifier_ui | |
| mesh[39].descriptor_ui | D000067996 |
| mesh[39].is_major_topic | False |
| mesh[39].qualifier_name | |
| mesh[39].descriptor_name | RAW 264.7 Cells |
| mesh[40].qualifier_ui | Q000378 |
| mesh[40].descriptor_ui | D051116 |
| mesh[40].is_major_topic | False |
| mesh[40].qualifier_name | metabolism |
| mesh[40].descriptor_name | Receptors, Scavenger |
| mesh[41].qualifier_ui | Q000378 |
| mesh[41].descriptor_ui | D013109 |
| mesh[41].is_major_topic | False |
| mesh[41].qualifier_name | metabolism |
| mesh[41].descriptor_name | Sphingomyelins |
| mesh[42].qualifier_ui | |
| mesh[42].descriptor_ui | D000818 |
| mesh[42].is_major_topic | False |
| mesh[42].qualifier_name | |
| mesh[42].descriptor_name | Animals |
| mesh[43].qualifier_ui | Q000502 |
| mesh[43].descriptor_ui | D001692 |
| mesh[43].is_major_topic | False |
| mesh[43].qualifier_name | physiology |
| mesh[43].descriptor_name | Biological Transport |
| mesh[44].qualifier_ui | Q000378 |
| mesh[44].descriptor_ui | D002402 |
| mesh[44].is_major_topic | False |
| mesh[44].qualifier_name | metabolism |
| mesh[44].descriptor_name | Cathepsin D |
| mesh[45].qualifier_ui | |
| mesh[45].descriptor_ui | D002460 |
| mesh[45].is_major_topic | False |
| mesh[45].qualifier_name | |
| mesh[45].descriptor_name | Cell Line |
| mesh[46].qualifier_ui | |
| mesh[46].descriptor_ui | D045744 |
| mesh[46].is_major_topic | False |
| mesh[46].qualifier_name | |
| mesh[46].descriptor_name | Cell Line, Tumor |
| mesh[47].qualifier_ui | Q000378 |
| mesh[47].descriptor_ui | D005776 |
| mesh[47].is_major_topic | False |
| mesh[47].qualifier_name | metabolism |
| mesh[47].descriptor_name | Gaucher Disease |
| mesh[48].qualifier_ui | Q000378 |
| mesh[48].descriptor_ui | D005962 |
| mesh[48].is_major_topic | False |
| mesh[48].qualifier_name | metabolism |
| mesh[48].descriptor_name | Glucosylceramidase |
| mesh[49].qualifier_ui | Q000378 |
| mesh[49].descriptor_ui | D005963 |
| mesh[49].is_major_topic | False |
| mesh[49].qualifier_name | metabolism |
| mesh[49].descriptor_name | Glucosylceramides |
| type | article |
| title | GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice |
| awards[0].id | https://openalex.org/G8860775887 |
| awards[0].funder_id | https://openalex.org/F4320321800 |
| awards[0].display_name | |
| awards[0].funder_award_id | BBOL-2007247202 |
| awards[0].funder_display_name | Nederlandse Organisatie voor Wetenschappelijk Onderzoek |
| biblio.issue | 5 |
| biblio.volume | 22 |
| biblio.last_page | 2532 |
| biblio.first_page | 2532 |
| grants[0].funder | https://openalex.org/F4320321800 |
| grants[0].award_id | BBOL-2007247202 |
| grants[0].funder_display_name | Nederlandse Organisatie voor Wetenschappelijk Onderzoek |
| topics[0].id | https://openalex.org/T10945 |
| topics[0].field.id | https://openalex.org/fields/27 |
| topics[0].field.display_name | Medicine |
| topics[0].score | 1.0 |
| topics[0].domain.id | https://openalex.org/domains/4 |
| topics[0].domain.display_name | Health Sciences |
| topics[0].subfield.id | https://openalex.org/subfields/2737 |
| topics[0].subfield.display_name | Physiology |
| topics[0].display_name | Lysosomal Storage Disorders Research |
| topics[1].id | https://openalex.org/T13415 |
| topics[1].field.id | https://openalex.org/fields/13 |
| topics[1].field.display_name | Biochemistry, Genetics and Molecular Biology |
| topics[1].score | 0.9886999726295471 |
| topics[1].domain.id | https://openalex.org/domains/1 |
| topics[1].domain.display_name | Life Sciences |
| topics[1].subfield.id | https://openalex.org/subfields/1314 |
| topics[1].subfield.display_name | Physiology |
| topics[1].display_name | Calcium signaling and nucleotide metabolism |
| topics[2].id | https://openalex.org/T10835 |
| topics[2].field.id | https://openalex.org/fields/16 |
| topics[2].field.display_name | Chemistry |
| topics[2].score | 0.9864000082015991 |
| topics[2].domain.id | https://openalex.org/domains/3 |
| topics[2].domain.display_name | Physical Sciences |
| topics[2].subfield.id | https://openalex.org/subfields/1605 |
| topics[2].subfield.display_name | Organic Chemistry |
| topics[2].display_name | Carbohydrate Chemistry and Synthesis |
| funders[0].id | https://openalex.org/F4320321800 |
| funders[0].ror | https://ror.org/04jsz6e67 |
| funders[0].display_name | Nederlandse Organisatie voor Wetenschappelijk Onderzoek |
| is_xpac | False |
| apc_list.value | 2300 |
| apc_list.currency | CHF |
| apc_list.value_usd | 2490 |
| apc_paid.value | 2300 |
| apc_paid.currency | CHF |
| apc_paid.value_usd | 2490 |
| concepts[0].id | https://openalex.org/C2778552423 |
| concepts[0].level | 4 |
| concepts[0].score | 0.8110322952270508 |
| concepts[0].wikidata | https://www.wikidata.org/wiki/Q14914279 |
| concepts[0].display_name | NPC1 |
| concepts[1].id | https://openalex.org/C2781457462 |
| concepts[1].level | 4 |
| concepts[1].score | 0.737089216709137 |
| concepts[1].wikidata | https://www.wikidata.org/wiki/Q16245045 |
| concepts[1].display_name | Acid sphingomyelinase |
| concepts[2].id | https://openalex.org/C2780635549 |
| concepts[2].level | 3 |
| concepts[2].score | 0.6479822397232056 |
| concepts[2].wikidata | https://www.wikidata.org/wiki/Q2067267 |
| concepts[2].display_name | Niemann–Pick disease, type C |
| concepts[3].id | https://openalex.org/C2779489717 |
| concepts[3].level | 3 |
| concepts[3].score | 0.637336790561676 |
| concepts[3].wikidata | https://www.wikidata.org/wiki/Q1419931 |
| concepts[3].display_name | Niemann–Pick disease |
| concepts[4].id | https://openalex.org/C61322309 |
| concepts[4].level | 3 |
| concepts[4].score | 0.6055920124053955 |
| concepts[4].wikidata | https://www.wikidata.org/wiki/Q423143 |
| concepts[4].display_name | Sphingomyelin |
| concepts[5].id | https://openalex.org/C2778163477 |
| concepts[5].level | 2 |
| concepts[5].score | 0.579653263092041 |
| concepts[5].wikidata | https://www.wikidata.org/wiki/Q43656 |
| concepts[5].display_name | Cholesterol |
| concepts[6].id | https://openalex.org/C61705674 |
| concepts[6].level | 2 |
| concepts[6].score | 0.5571795105934143 |
| concepts[6].wikidata | https://www.wikidata.org/wiki/Q410395 |
| concepts[6].display_name | Sphingolipid |
| concepts[7].id | https://openalex.org/C134018914 |
| concepts[7].level | 1 |
| concepts[7].score | 0.4792328178882599 |
| concepts[7].wikidata | https://www.wikidata.org/wiki/Q162606 |
| concepts[7].display_name | Endocrinology |
| concepts[8].id | https://openalex.org/C2777851122 |
| concepts[8].level | 3 |
| concepts[8].score | 0.4778197109699249 |
| concepts[8].wikidata | https://www.wikidata.org/wiki/Q424213 |
| concepts[8].display_name | Ceramide |
| concepts[9].id | https://openalex.org/C86803240 |
| concepts[9].level | 0 |
| concepts[9].score | 0.4775196611881256 |
| concepts[9].wikidata | https://www.wikidata.org/wiki/Q420 |
| concepts[9].display_name | Biology |
| concepts[10].id | https://openalex.org/C2777331542 |
| concepts[10].level | 2 |
| concepts[10].score | 0.4702523946762085 |
| concepts[10].wikidata | https://www.wikidata.org/wiki/Q419287 |
| concepts[10].display_name | Ganglioside |
| concepts[11].id | https://openalex.org/C126322002 |
| concepts[11].level | 1 |
| concepts[11].score | 0.4472278356552124 |
| concepts[11].wikidata | https://www.wikidata.org/wiki/Q11180 |
| concepts[11].display_name | Internal medicine |
| concepts[12].id | https://openalex.org/C2781070647 |
| concepts[12].level | 4 |
| concepts[12].score | 0.4221493601799011 |
| concepts[12].wikidata | https://www.wikidata.org/wiki/Q413230 |
| concepts[12].display_name | Sphingomyelin phosphodiesterase |
| concepts[13].id | https://openalex.org/C2776200302 |
| concepts[13].level | 3 |
| concepts[13].score | 0.4115239977836609 |
| concepts[13].wikidata | https://www.wikidata.org/wiki/Q827450 |
| concepts[13].display_name | Hepatocyte |
| concepts[14].id | https://openalex.org/C185592680 |
| concepts[14].level | 0 |
| concepts[14].score | 0.39647430181503296 |
| concepts[14].wikidata | https://www.wikidata.org/wiki/Q2329 |
| concepts[14].display_name | Chemistry |
| concepts[15].id | https://openalex.org/C55493867 |
| concepts[15].level | 1 |
| concepts[15].score | 0.38412874937057495 |
| concepts[15].wikidata | https://www.wikidata.org/wiki/Q7094 |
| concepts[15].display_name | Biochemistry |
| concepts[16].id | https://openalex.org/C102747710 |
| concepts[16].level | 3 |
| concepts[16].score | 0.23375895619392395 |
| concepts[16].wikidata | https://www.wikidata.org/wiki/Q824237 |
| concepts[16].display_name | Endosome |
| concepts[17].id | https://openalex.org/C71924100 |
| concepts[17].level | 0 |
| concepts[17].score | 0.13669165968894958 |
| concepts[17].wikidata | https://www.wikidata.org/wiki/Q11190 |
| concepts[17].display_name | Medicine |
| concepts[18].id | https://openalex.org/C79879829 |
| concepts[18].level | 2 |
| concepts[18].score | 0.11061057448387146 |
| concepts[18].wikidata | https://www.wikidata.org/wiki/Q5571762 |
| concepts[18].display_name | Intracellular |
| concepts[19].id | https://openalex.org/C202751555 |
| concepts[19].level | 2 |
| concepts[19].score | 0.0 |
| concepts[19].wikidata | https://www.wikidata.org/wiki/Q221681 |
| concepts[19].display_name | In vitro |
| concepts[20].id | https://openalex.org/C190283241 |
| concepts[20].level | 2 |
| concepts[20].score | 0.0 |
| concepts[20].wikidata | https://www.wikidata.org/wiki/Q14599311 |
| concepts[20].display_name | Apoptosis |
| keywords[0].id | https://openalex.org/keywords/npc1 |
| keywords[0].score | 0.8110322952270508 |
| keywords[0].display_name | NPC1 |
| keywords[1].id | https://openalex.org/keywords/acid-sphingomyelinase |
| keywords[1].score | 0.737089216709137 |
| keywords[1].display_name | Acid sphingomyelinase |
| keywords[2].id | https://openalex.org/keywords/niemann–pick-disease-type-c |
| keywords[2].score | 0.6479822397232056 |
| keywords[2].display_name | Niemann–Pick disease, type C |
| keywords[3].id | https://openalex.org/keywords/niemann–pick-disease |
| keywords[3].score | 0.637336790561676 |
| keywords[3].display_name | Niemann–Pick disease |
| keywords[4].id | https://openalex.org/keywords/sphingomyelin |
| keywords[4].score | 0.6055920124053955 |
| keywords[4].display_name | Sphingomyelin |
| keywords[5].id | https://openalex.org/keywords/cholesterol |
| keywords[5].score | 0.579653263092041 |
| keywords[5].display_name | Cholesterol |
| keywords[6].id | https://openalex.org/keywords/sphingolipid |
| keywords[6].score | 0.5571795105934143 |
| keywords[6].display_name | Sphingolipid |
| keywords[7].id | https://openalex.org/keywords/endocrinology |
| keywords[7].score | 0.4792328178882599 |
| keywords[7].display_name | Endocrinology |
| keywords[8].id | https://openalex.org/keywords/ceramide |
| keywords[8].score | 0.4778197109699249 |
| keywords[8].display_name | Ceramide |
| keywords[9].id | https://openalex.org/keywords/biology |
| keywords[9].score | 0.4775196611881256 |
| keywords[9].display_name | Biology |
| keywords[10].id | https://openalex.org/keywords/ganglioside |
| keywords[10].score | 0.4702523946762085 |
| keywords[10].display_name | Ganglioside |
| keywords[11].id | https://openalex.org/keywords/internal-medicine |
| keywords[11].score | 0.4472278356552124 |
| keywords[11].display_name | Internal medicine |
| keywords[12].id | https://openalex.org/keywords/sphingomyelin-phosphodiesterase |
| keywords[12].score | 0.4221493601799011 |
| keywords[12].display_name | Sphingomyelin phosphodiesterase |
| keywords[13].id | https://openalex.org/keywords/hepatocyte |
| keywords[13].score | 0.4115239977836609 |
| keywords[13].display_name | Hepatocyte |
| keywords[14].id | https://openalex.org/keywords/chemistry |
| keywords[14].score | 0.39647430181503296 |
| keywords[14].display_name | Chemistry |
| keywords[15].id | https://openalex.org/keywords/biochemistry |
| keywords[15].score | 0.38412874937057495 |
| keywords[15].display_name | Biochemistry |
| keywords[16].id | https://openalex.org/keywords/endosome |
| keywords[16].score | 0.23375895619392395 |
| keywords[16].display_name | Endosome |
| keywords[17].id | https://openalex.org/keywords/medicine |
| keywords[17].score | 0.13669165968894958 |
| keywords[17].display_name | Medicine |
| keywords[18].id | https://openalex.org/keywords/intracellular |
| keywords[18].score | 0.11061057448387146 |
| keywords[18].display_name | Intracellular |
| language | en |
| locations[0].id | doi:10.3390/ijms22052532 |
| locations[0].is_oa | True |
| locations[0].source.id | https://openalex.org/S10623703 |
| locations[0].source.issn | 1422-0067, 1661-6596 |
| locations[0].source.type | journal |
| locations[0].source.is_oa | True |
| locations[0].source.issn_l | 1422-0067 |
| locations[0].source.is_core | True |
| locations[0].source.is_in_doaj | False |
| locations[0].source.display_name | International Journal of Molecular Sciences |
| locations[0].source.host_organization | https://openalex.org/P4310310987 |
| locations[0].source.host_organization_name | Multidisciplinary Digital Publishing Institute |
| locations[0].source.host_organization_lineage | https://openalex.org/P4310310987 |
| locations[0].source.host_organization_lineage_names | Multidisciplinary Digital Publishing Institute |
| locations[0].license | cc-by |
| locations[0].pdf_url | https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655 |
| locations[0].version | publishedVersion |
| locations[0].raw_type | journal-article |
| locations[0].license_id | https://openalex.org/licenses/cc-by |
| locations[0].is_accepted | True |
| locations[0].is_published | True |
| locations[0].raw_source_name | International Journal of Molecular Sciences |
| locations[0].landing_page_url | https://doi.org/10.3390/ijms22052532 |
| locations[1].id | pmid:33802460 |
| locations[1].is_oa | False |
| locations[1].source.id | https://openalex.org/S4306525036 |
| locations[1].source.issn | |
| locations[1].source.type | repository |
| locations[1].source.is_oa | False |
| locations[1].source.issn_l | |
| locations[1].source.is_core | False |
| locations[1].source.is_in_doaj | False |
| locations[1].source.display_name | PubMed |
| locations[1].source.host_organization | https://openalex.org/I1299303238 |
| locations[1].source.host_organization_name | National Institutes of Health |
| locations[1].source.host_organization_lineage | https://openalex.org/I1299303238 |
| locations[1].license | |
| locations[1].pdf_url | |
| locations[1].version | publishedVersion |
| locations[1].raw_type | |
| locations[1].license_id | |
| locations[1].is_accepted | True |
| locations[1].is_published | True |
| locations[1].raw_source_name | International journal of molecular sciences |
| locations[1].landing_page_url | https://pubmed.ncbi.nlm.nih.gov/33802460 |
| locations[2].id | pmh:amcpub:oai:pure.amc.nl:publications/0762b8f7-d6e3-40fa-97db-6d05bf814e52 |
| locations[2].is_oa | True |
| locations[2].source.id | https://openalex.org/S4306401843 |
| locations[2].source.issn | |
| locations[2].source.type | repository |
| locations[2].source.is_oa | False |
| locations[2].source.issn_l | |
| locations[2].source.is_core | False |
| locations[2].source.is_in_doaj | False |
| locations[2].source.display_name | Data Archiving and Networked Services (DANS) |
| locations[2].source.host_organization | https://openalex.org/I1322597698 |
| locations[2].source.host_organization_name | Royal Netherlands Academy of Arts and Sciences |
| locations[2].source.host_organization_lineage | https://openalex.org/I1322597698 |
| locations[2].license | other-oa |
| locations[2].pdf_url | |
| locations[2].version | submittedVersion |
| locations[2].raw_type | info:eu-repo/semantics/article |
| locations[2].license_id | https://openalex.org/licenses/other-oa |
| locations[2].is_accepted | False |
| locations[2].is_published | False |
| locations[2].raw_source_name | International journal of molecular sciences, 22(5):2532, 1 - 15. Multidisciplinary Digital Publishing Institute (MDPI) |
| locations[2].landing_page_url | https://pure.amc.nl/en/publications/gcase-and-limp2-abnormalities-in-the-liver-of-niemann-pick-type-c-mice(0762b8f7-d6e3-40fa-97db-6d05bf814e52).html |
| locations[3].id | pmh:vumc:oai:pure.atira.dk:publications/c14b4b58-a352-4207-a8b3-0f94b00e154d |
| locations[3].is_oa | True |
| locations[3].source.id | https://openalex.org/S4306401843 |
| locations[3].source.issn | |
| locations[3].source.type | repository |
| locations[3].source.is_oa | False |
| locations[3].source.issn_l | |
| locations[3].source.is_core | False |
| locations[3].source.is_in_doaj | False |
| locations[3].source.display_name | Data Archiving and Networked Services (DANS) |
| locations[3].source.host_organization | https://openalex.org/I1322597698 |
| locations[3].source.host_organization_name | Royal Netherlands Academy of Arts and Sciences |
| locations[3].source.host_organization_lineage | https://openalex.org/I1322597698 |
| locations[3].license | other-oa |
| locations[3].pdf_url | |
| locations[3].version | submittedVersion |
| locations[3].raw_type | info:eu-repo/semantics/article |
| locations[3].license_id | https://openalex.org/licenses/other-oa |
| locations[3].is_accepted | False |
| locations[3].is_published | False |
| locations[3].raw_source_name | International Journal of Molecular Sciences, 22(5):2532, 1 - 15. Multidisciplinary Digital Publishing Institute (MDPI) |
| locations[3].landing_page_url | https://research.vumc.nl/en/publications/c14b4b58-a352-4207-a8b3-0f94b00e154d |
| locations[4].id | pmh:oai:run.unl.pt:10362/124966 |
| locations[4].is_oa | True |
| locations[4].source.id | https://openalex.org/S4306400678 |
| locations[4].source.issn | |
| locations[4].source.type | repository |
| locations[4].source.is_oa | False |
| locations[4].source.issn_l | |
| locations[4].source.is_core | False |
| locations[4].source.is_in_doaj | False |
| locations[4].source.display_name | Universidade Nova de Lisboa's Repository (Universidade Nova de Lisboa) |
| locations[4].source.host_organization | https://openalex.org/I83558840 |
| locations[4].source.host_organization_name | Universidade Nova de Lisboa |
| locations[4].source.host_organization_lineage | https://openalex.org/I83558840 |
| locations[4].license | other-oa |
| locations[4].pdf_url | |
| locations[4].version | submittedVersion |
| locations[4].raw_type | |
| locations[4].license_id | https://openalex.org/licenses/other-oa |
| locations[4].is_accepted | False |
| locations[4].is_published | False |
| locations[4].raw_source_name | |
| locations[4].landing_page_url | http://hdl.handle.net/10362/124966 |
| locations[5].id | pmh:oai:scholarlypublications.universiteitleiden.nl:item_3254570 |
| locations[5].is_oa | True |
| locations[5].source.id | https://openalex.org/S10623703 |
| locations[5].source.issn | 1422-0067, 1661-6596 |
| locations[5].source.type | journal |
| locations[5].source.is_oa | True |
| locations[5].source.issn_l | 1422-0067 |
| locations[5].source.is_core | True |
| locations[5].source.is_in_doaj | False |
| locations[5].source.display_name | International Journal of Molecular Sciences |
| locations[5].source.host_organization | https://openalex.org/P4310310987 |
| locations[5].source.host_organization_name | Multidisciplinary Digital Publishing Institute |
| locations[5].source.host_organization_lineage | https://openalex.org/P4310310987 |
| locations[5].source.host_organization_lineage_names | Multidisciplinary Digital Publishing Institute |
| locations[5].license | cc-by |
| locations[5].pdf_url | |
| locations[5].version | submittedVersion |
| locations[5].raw_type | Article / Letter to editor |
| locations[5].license_id | https://openalex.org/licenses/cc-by |
| locations[5].is_accepted | False |
| locations[5].is_published | False |
| locations[5].raw_source_name | International Journal of Molecular Sciences |
| locations[5].landing_page_url | https://hdl.handle.net/1887/3254570 |
| locations[6].id | pmh:oai:doaj.org/article:70f7f422666d4419839e25546388fa0d |
| locations[6].is_oa | True |
| locations[6].source.id | https://openalex.org/S4306401280 |
| locations[6].source.issn | |
| locations[6].source.type | repository |
| locations[6].source.is_oa | False |
| locations[6].source.issn_l | |
| locations[6].source.is_core | False |
| locations[6].source.is_in_doaj | False |
| locations[6].source.display_name | DOAJ (DOAJ: Directory of Open Access Journals) |
| locations[6].source.host_organization | |
| locations[6].source.host_organization_name | |
| locations[6].license | cc-by-sa |
| locations[6].pdf_url | |
| locations[6].version | submittedVersion |
| locations[6].raw_type | article |
| locations[6].license_id | https://openalex.org/licenses/cc-by-sa |
| locations[6].is_accepted | False |
| locations[6].is_published | False |
| locations[6].raw_source_name | International Journal of Molecular Sciences, Vol 22, Iss 2532, p 2532 (2021) |
| locations[6].landing_page_url | https://doaj.org/article/70f7f422666d4419839e25546388fa0d |
| locations[7].id | pmh:oai:mdpi.com:/1422-0067/22/5/2532/ |
| locations[7].is_oa | True |
| locations[7].source.id | https://openalex.org/S4306400947 |
| locations[7].source.issn | |
| locations[7].source.type | repository |
| locations[7].source.is_oa | True |
| locations[7].source.issn_l | |
| locations[7].source.is_core | False |
| locations[7].source.is_in_doaj | False |
| locations[7].source.display_name | MDPI (MDPI AG) |
| locations[7].source.host_organization | https://openalex.org/I4210097602 |
| locations[7].source.host_organization_name | Multidisciplinary Digital Publishing Institute (Switzerland) |
| locations[7].source.host_organization_lineage | https://openalex.org/I4210097602 |
| locations[7].license | cc-by |
| locations[7].pdf_url | |
| locations[7].version | submittedVersion |
| locations[7].raw_type | Text |
| locations[7].license_id | https://openalex.org/licenses/cc-by |
| locations[7].is_accepted | False |
| locations[7].is_published | False |
| locations[7].raw_source_name | International Journal of Molecular Sciences; Volume 22; Issue 5; Pages: 2532 |
| locations[7].landing_page_url | https://dx.doi.org/10.3390/ijms22052532 |
| locations[8].id | pmh:ul:oai:scholarlypublications.universiteitleiden.nl:item_3254570 |
| locations[8].is_oa | True |
| locations[8].source.id | https://openalex.org/S4306401843 |
| locations[8].source.issn | |
| locations[8].source.type | repository |
| locations[8].source.is_oa | False |
| locations[8].source.issn_l | |
| locations[8].source.is_core | False |
| locations[8].source.is_in_doaj | False |
| locations[8].source.display_name | Data Archiving and Networked Services (DANS) |
| locations[8].source.host_organization | https://openalex.org/I1322597698 |
| locations[8].source.host_organization_name | Royal Netherlands Academy of Arts and Sciences |
| locations[8].source.host_organization_lineage | https://openalex.org/I1322597698 |
| locations[8].license | cc-by |
| locations[8].pdf_url | |
| locations[8].version | submittedVersion |
| locations[8].raw_type | info:eu-repo/semantics/article |
| locations[8].license_id | https://openalex.org/licenses/cc-by |
| locations[8].is_accepted | False |
| locations[8].is_published | False |
| locations[8].raw_source_name | International Journal of Molecular Sciences, 22(5). MDPI} {AG |
| locations[8].landing_page_url | http://hdl.handle.net/1887/3254570 |
| locations[9].id | pmh:oai:pubmedcentral.nih.gov:7959463 |
| locations[9].is_oa | True |
| locations[9].source.id | https://openalex.org/S2764455111 |
| locations[9].source.issn | |
| locations[9].source.type | repository |
| locations[9].source.is_oa | False |
| locations[9].source.issn_l | |
| locations[9].source.is_core | False |
| locations[9].source.is_in_doaj | False |
| locations[9].source.display_name | PubMed Central |
| locations[9].source.host_organization | https://openalex.org/I1299303238 |
| locations[9].source.host_organization_name | National Institutes of Health |
| locations[9].source.host_organization_lineage | https://openalex.org/I1299303238 |
| locations[9].license | other-oa |
| locations[9].pdf_url | |
| locations[9].version | submittedVersion |
| locations[9].raw_type | Text |
| locations[9].license_id | https://openalex.org/licenses/other-oa |
| locations[9].is_accepted | False |
| locations[9].is_published | False |
| locations[9].raw_source_name | Int J Mol Sci |
| locations[9].landing_page_url | https://www.ncbi.nlm.nih.gov/pmc/articles/7959463 |
| indexed_in | crossref, doaj, pubmed |
| authorships[0].author.id | https://openalex.org/A5035632542 |
| authorships[0].author.orcid | https://orcid.org/0000-0002-6458-1479 |
| authorships[0].author.display_name | Martijn J. C. van der Lienden |
| authorships[0].countries | NL |
| authorships[0].affiliations[0].institution_ids | https://openalex.org/I121797337 |
| authorships[0].affiliations[0].raw_affiliation_string | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| authorships[0].institutions[0].id | https://openalex.org/I121797337 |
| authorships[0].institutions[0].ror | https://ror.org/027bh9e22 |
| authorships[0].institutions[0].type | education |
| authorships[0].institutions[0].lineage | https://openalex.org/I121797337 |
| authorships[0].institutions[0].country_code | NL |
| authorships[0].institutions[0].display_name | Leiden University |
| authorships[0].author_position | first |
| authorships[0].raw_author_name | Martijn J. C. van der Lienden |
| authorships[0].is_corresponding | False |
| authorships[0].raw_affiliation_strings | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| authorships[1].author.id | https://openalex.org/A5070030883 |
| authorships[1].author.orcid | https://orcid.org/0000-0002-0997-6777 |
| authorships[1].author.display_name | Jan Aten |
| authorships[1].countries | NL |
| authorships[1].affiliations[0].institution_ids | https://openalex.org/I4210151833, https://openalex.org/I887064364 |
| authorships[1].affiliations[0].raw_affiliation_string | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[1].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[1].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[1].institutions[0].type | healthcare |
| authorships[1].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[1].institutions[0].country_code | NL |
| authorships[1].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[1].institutions[1].id | https://openalex.org/I887064364 |
| authorships[1].institutions[1].ror | https://ror.org/04dkp9463 |
| authorships[1].institutions[1].type | education |
| authorships[1].institutions[1].lineage | https://openalex.org/I887064364 |
| authorships[1].institutions[1].country_code | NL |
| authorships[1].institutions[1].display_name | University of Amsterdam |
| authorships[1].author_position | middle |
| authorships[1].raw_author_name | Jan Aten |
| authorships[1].is_corresponding | False |
| authorships[1].raw_affiliation_strings | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[2].author.id | https://openalex.org/A5022416494 |
| authorships[2].author.orcid | https://orcid.org/0000-0001-9674-3017 |
| authorships[2].author.display_name | André R. A. Marques |
| authorships[2].countries | PT |
| authorships[2].affiliations[0].institution_ids | https://openalex.org/I83558840 |
| authorships[2].affiliations[0].raw_affiliation_string | Chronic Diseases Research Centre, Universidade NOVA de Lisboa, 1150-082 Lisbon, Portugal |
| authorships[2].institutions[0].id | https://openalex.org/I83558840 |
| authorships[2].institutions[0].ror | https://ror.org/02xankh89 |
| authorships[2].institutions[0].type | education |
| authorships[2].institutions[0].lineage | https://openalex.org/I83558840 |
| authorships[2].institutions[0].country_code | PT |
| authorships[2].institutions[0].display_name | Universidade Nova de Lisboa |
| authorships[2].author_position | middle |
| authorships[2].raw_author_name | André R. A. Marques |
| authorships[2].is_corresponding | False |
| authorships[2].raw_affiliation_strings | Chronic Diseases Research Centre, Universidade NOVA de Lisboa, 1150-082 Lisbon, Portugal |
| authorships[3].author.id | https://openalex.org/A5071364020 |
| authorships[3].author.orcid | |
| authorships[3].author.display_name | Ingeborg S. E. Waas |
| authorships[3].countries | NL |
| authorships[3].affiliations[0].institution_ids | https://openalex.org/I4210151833, https://openalex.org/I887064364 |
| authorships[3].affiliations[0].raw_affiliation_string | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[3].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[3].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[3].institutions[0].type | healthcare |
| authorships[3].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[3].institutions[0].country_code | NL |
| authorships[3].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[3].institutions[1].id | https://openalex.org/I887064364 |
| authorships[3].institutions[1].ror | https://ror.org/04dkp9463 |
| authorships[3].institutions[1].type | education |
| authorships[3].institutions[1].lineage | https://openalex.org/I887064364 |
| authorships[3].institutions[1].country_code | NL |
| authorships[3].institutions[1].display_name | University of Amsterdam |
| authorships[3].author_position | middle |
| authorships[3].raw_author_name | Ingeborg S. E. Waas |
| authorships[3].is_corresponding | False |
| authorships[3].raw_affiliation_strings | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[4].author.id | https://openalex.org/A5053345105 |
| authorships[4].author.orcid | https://orcid.org/0000-0001-9156-6541 |
| authorships[4].author.display_name | Per Larsen |
| authorships[4].countries | NL |
| authorships[4].affiliations[0].institution_ids | https://openalex.org/I4210151833, https://openalex.org/I887064364 |
| authorships[4].affiliations[0].raw_affiliation_string | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[4].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[4].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[4].institutions[0].type | healthcare |
| authorships[4].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[4].institutions[0].country_code | NL |
| authorships[4].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[4].institutions[1].id | https://openalex.org/I887064364 |
| authorships[4].institutions[1].ror | https://ror.org/04dkp9463 |
| authorships[4].institutions[1].type | education |
| authorships[4].institutions[1].lineage | https://openalex.org/I887064364 |
| authorships[4].institutions[1].country_code | NL |
| authorships[4].institutions[1].display_name | University of Amsterdam |
| authorships[4].author_position | middle |
| authorships[4].raw_author_name | Per W. B. Larsen |
| authorships[4].is_corresponding | False |
| authorships[4].raw_affiliation_strings | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[5].author.id | https://openalex.org/A5001169077 |
| authorships[5].author.orcid | |
| authorships[5].author.display_name | Nike Claessen |
| authorships[5].countries | NL |
| authorships[5].affiliations[0].institution_ids | https://openalex.org/I4210151833, https://openalex.org/I887064364 |
| authorships[5].affiliations[0].raw_affiliation_string | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[5].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[5].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[5].institutions[0].type | healthcare |
| authorships[5].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[5].institutions[0].country_code | NL |
| authorships[5].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[5].institutions[1].id | https://openalex.org/I887064364 |
| authorships[5].institutions[1].ror | https://ror.org/04dkp9463 |
| authorships[5].institutions[1].type | education |
| authorships[5].institutions[1].lineage | https://openalex.org/I887064364 |
| authorships[5].institutions[1].country_code | NL |
| authorships[5].institutions[1].display_name | University of Amsterdam |
| authorships[5].author_position | middle |
| authorships[5].raw_author_name | Nike Claessen |
| authorships[5].is_corresponding | False |
| authorships[5].raw_affiliation_strings | Department of Pathology, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[6].author.id | https://openalex.org/A5032689562 |
| authorships[6].author.orcid | https://orcid.org/0000-0001-6392-3417 |
| authorships[6].author.display_name | Nicole N. van der Wel |
| authorships[6].countries | NL |
| authorships[6].affiliations[0].institution_ids | https://openalex.org/I4210151833 |
| authorships[6].affiliations[0].raw_affiliation_string | Electron Microscopy Center Amsterdam, Department of Medical Biology, Amsterdam UMC, 1100 DD Amsterdam, The Netherlands |
| authorships[6].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[6].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[6].institutions[0].type | healthcare |
| authorships[6].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[6].institutions[0].country_code | NL |
| authorships[6].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[6].author_position | middle |
| authorships[6].raw_author_name | Nicole N. van der Wel |
| authorships[6].is_corresponding | False |
| authorships[6].raw_affiliation_strings | Electron Microscopy Center Amsterdam, Department of Medical Biology, Amsterdam UMC, 1100 DD Amsterdam, The Netherlands |
| authorships[7].author.id | https://openalex.org/A5026410723 |
| authorships[7].author.orcid | https://orcid.org/0000-0003-3023-1128 |
| authorships[7].author.display_name | Roelof Ottenhoff |
| authorships[7].countries | NL |
| authorships[7].affiliations[0].institution_ids | https://openalex.org/I4210151833, https://openalex.org/I887064364 |
| authorships[7].affiliations[0].raw_affiliation_string | Department of Medical Biochemistry, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[7].institutions[0].id | https://openalex.org/I4210151833 |
| authorships[7].institutions[0].ror | https://ror.org/05grdyy37 |
| authorships[7].institutions[0].type | healthcare |
| authorships[7].institutions[0].lineage | https://openalex.org/I4210151833 |
| authorships[7].institutions[0].country_code | NL |
| authorships[7].institutions[0].display_name | Amsterdam University Medical Centers |
| authorships[7].institutions[1].id | https://openalex.org/I887064364 |
| authorships[7].institutions[1].ror | https://ror.org/04dkp9463 |
| authorships[7].institutions[1].type | education |
| authorships[7].institutions[1].lineage | https://openalex.org/I887064364 |
| authorships[7].institutions[1].country_code | NL |
| authorships[7].institutions[1].display_name | University of Amsterdam |
| authorships[7].author_position | middle |
| authorships[7].raw_author_name | Roelof Ottenhoff |
| authorships[7].is_corresponding | False |
| authorships[7].raw_affiliation_strings | Department of Medical Biochemistry, Amsterdam UMC, University of Amsterdam, 1100 DD Amsterdam, The Netherlands |
| authorships[8].author.id | https://openalex.org/A5050568897 |
| authorships[8].author.orcid | https://orcid.org/0000-0001-7751-4112 |
| authorships[8].author.display_name | Marco van Eijk |
| authorships[8].countries | NL |
| authorships[8].affiliations[0].institution_ids | https://openalex.org/I121797337 |
| authorships[8].affiliations[0].raw_affiliation_string | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| authorships[8].institutions[0].id | https://openalex.org/I121797337 |
| authorships[8].institutions[0].ror | https://ror.org/027bh9e22 |
| authorships[8].institutions[0].type | education |
| authorships[8].institutions[0].lineage | https://openalex.org/I121797337 |
| authorships[8].institutions[0].country_code | NL |
| authorships[8].institutions[0].display_name | Leiden University |
| authorships[8].author_position | middle |
| authorships[8].raw_author_name | Marco van Eijk |
| authorships[8].is_corresponding | False |
| authorships[8].raw_affiliation_strings | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| authorships[9].author.id | https://openalex.org/A5013935159 |
| authorships[9].author.orcid | https://orcid.org/0000-0001-8168-2565 |
| authorships[9].author.display_name | Johannes M. F. G. Aerts |
| authorships[9].countries | NL |
| authorships[9].affiliations[0].institution_ids | https://openalex.org/I121797337 |
| authorships[9].affiliations[0].raw_affiliation_string | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| authorships[9].institutions[0].id | https://openalex.org/I121797337 |
| authorships[9].institutions[0].ror | https://ror.org/027bh9e22 |
| authorships[9].institutions[0].type | education |
| authorships[9].institutions[0].lineage | https://openalex.org/I121797337 |
| authorships[9].institutions[0].country_code | NL |
| authorships[9].institutions[0].display_name | Leiden University |
| authorships[9].author_position | last |
| authorships[9].raw_author_name | Johannes M. F. G. Aerts |
| authorships[9].is_corresponding | True |
| authorships[9].raw_affiliation_strings | Department Medical Biochemistry, Leiden University, 2333 CC Leiden, The Netherlands |
| has_content.pdf | True |
| has_content.grobid_xml | True |
| is_paratext | False |
| open_access.is_oa | True |
| open_access.oa_url | https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655 |
| open_access.oa_status | gold |
| open_access.any_repository_has_fulltext | False |
| created_date | 2025-10-10T00:00:00 |
| display_name | GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice |
| has_fulltext | True |
| is_retracted | False |
| updated_date | 2025-11-06T03:46:38.306776 |
| primary_topic.id | https://openalex.org/T10945 |
| primary_topic.field.id | https://openalex.org/fields/27 |
| primary_topic.field.display_name | Medicine |
| primary_topic.score | 1.0 |
| primary_topic.domain.id | https://openalex.org/domains/4 |
| primary_topic.domain.display_name | Health Sciences |
| primary_topic.subfield.id | https://openalex.org/subfields/2737 |
| primary_topic.subfield.display_name | Physiology |
| primary_topic.display_name | Lysosomal Storage Disorders Research |
| related_works | https://openalex.org/W2008994625, https://openalex.org/W2889235373, https://openalex.org/W2164062211, https://openalex.org/W3021178475, https://openalex.org/W2024286256, https://openalex.org/W2015627456, https://openalex.org/W3134294420, https://openalex.org/W593268778, https://openalex.org/W1967723482, https://openalex.org/W1789442069 |
| cited_by_count | 35 |
| counts_by_year[0].year | 2025 |
| counts_by_year[0].cited_by_count | 1 |
| counts_by_year[1].year | 2024 |
| counts_by_year[1].cited_by_count | 1 |
| counts_by_year[2].year | 2023 |
| counts_by_year[2].cited_by_count | 2 |
| counts_by_year[3].year | 2022 |
| counts_by_year[3].cited_by_count | 2 |
| counts_by_year[4].year | 2021 |
| counts_by_year[4].cited_by_count | 29 |
| locations_count | 10 |
| best_oa_location.id | doi:10.3390/ijms22052532 |
| best_oa_location.is_oa | True |
| best_oa_location.source.id | https://openalex.org/S10623703 |
| best_oa_location.source.issn | 1422-0067, 1661-6596 |
| best_oa_location.source.type | journal |
| best_oa_location.source.is_oa | True |
| best_oa_location.source.issn_l | 1422-0067 |
| best_oa_location.source.is_core | True |
| best_oa_location.source.is_in_doaj | False |
| best_oa_location.source.display_name | International Journal of Molecular Sciences |
| best_oa_location.source.host_organization | https://openalex.org/P4310310987 |
| best_oa_location.source.host_organization_name | Multidisciplinary Digital Publishing Institute |
| best_oa_location.source.host_organization_lineage | https://openalex.org/P4310310987 |
| best_oa_location.source.host_organization_lineage_names | Multidisciplinary Digital Publishing Institute |
| best_oa_location.license | cc-by |
| best_oa_location.pdf_url | https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655 |
| best_oa_location.version | publishedVersion |
| best_oa_location.raw_type | journal-article |
| best_oa_location.license_id | https://openalex.org/licenses/cc-by |
| best_oa_location.is_accepted | True |
| best_oa_location.is_published | True |
| best_oa_location.raw_source_name | International Journal of Molecular Sciences |
| best_oa_location.landing_page_url | https://doi.org/10.3390/ijms22052532 |
| primary_location.id | doi:10.3390/ijms22052532 |
| primary_location.is_oa | True |
| primary_location.source.id | https://openalex.org/S10623703 |
| primary_location.source.issn | 1422-0067, 1661-6596 |
| primary_location.source.type | journal |
| primary_location.source.is_oa | True |
| primary_location.source.issn_l | 1422-0067 |
| primary_location.source.is_core | True |
| primary_location.source.is_in_doaj | False |
| primary_location.source.display_name | International Journal of Molecular Sciences |
| primary_location.source.host_organization | https://openalex.org/P4310310987 |
| primary_location.source.host_organization_name | Multidisciplinary Digital Publishing Institute |
| primary_location.source.host_organization_lineage | https://openalex.org/P4310310987 |
| primary_location.source.host_organization_lineage_names | Multidisciplinary Digital Publishing Institute |
| primary_location.license | cc-by |
| primary_location.pdf_url | https://www.mdpi.com/1422-0067/22/5/2532/pdf?version=1615166655 |
| primary_location.version | publishedVersion |
| primary_location.raw_type | journal-article |
| primary_location.license_id | https://openalex.org/licenses/cc-by |
| primary_location.is_accepted | True |
| primary_location.is_published | True |
| primary_location.raw_source_name | International Journal of Molecular Sciences |
| primary_location.landing_page_url | https://doi.org/10.3390/ijms22052532 |
| publication_date | 2021-03-03 |
| publication_year | 2021 |
| referenced_works | https://openalex.org/W2146156622, https://openalex.org/W2068262899, https://openalex.org/W2013325749, https://openalex.org/W2194088231, https://openalex.org/W1981615019, https://openalex.org/W1964623101, https://openalex.org/W2031491550, https://openalex.org/W2206291032, https://openalex.org/W2329337854, https://openalex.org/W2208335363, https://openalex.org/W1775418466, https://openalex.org/W2079133058, https://openalex.org/W1526003561, https://openalex.org/W1491957044, https://openalex.org/W2966569611, https://openalex.org/W2954907128, https://openalex.org/W2925222829, https://openalex.org/W2144456776, https://openalex.org/W2097813811, https://openalex.org/W2149584682, https://openalex.org/W2346657977, https://openalex.org/W2905755247, https://openalex.org/W2991494777, https://openalex.org/W1971217129, https://openalex.org/W2239401977, https://openalex.org/W2163527490, https://openalex.org/W2080225700, https://openalex.org/W2934235733, https://openalex.org/W7034647589, https://openalex.org/W2164687616, https://openalex.org/W2991456852, https://openalex.org/W2077499130, https://openalex.org/W2062956001, https://openalex.org/W2134637559, https://openalex.org/W2778101470, https://openalex.org/W2164906860, https://openalex.org/W2119327642, https://openalex.org/W2028049627, https://openalex.org/W2017112394, https://openalex.org/W2095855268, https://openalex.org/W2035849215 |
| referenced_works_count | 41 |
| abstract_inverted_index.B | 90 |
| abstract_inverted_index.C | 6 |
| abstract_inverted_index.a | 92, 106, 207 |
| abstract_inverted_index.D, | 136 |
| abstract_inverted_index.GD | 71 |
| abstract_inverted_index.In | 49, 114, 127 |
| abstract_inverted_index.an | 43 |
| abstract_inverted_index.as | 141, 143 |
| abstract_inverted_index.be | 120 |
| abstract_inverted_index.by | 10, 19, 160 |
| abstract_inverted_index.in | 35, 170, 183, 191, 215 |
| abstract_inverted_index.is | 8, 17, 180 |
| abstract_inverted_index.of | 23, 51, 64, 67, 70, 94, 101, 132, 146, 168, 186, 221 |
| abstract_inverted_index.on | 78 |
| abstract_inverted_index.to | 29, 98, 119, 196, 199 |
| abstract_inverted_index.we | 60 |
| abstract_inverted_index.NPC | 40 |
| abstract_inverted_index.The | 0, 201 |
| abstract_inverted_index.and | 25, 46, 76, 84, 111, 150, 226 |
| abstract_inverted_index.are | 158 |
| abstract_inverted_index.not | 195 |
| abstract_inverted_index.the | 54, 62, 79, 124, 155, 184, 187, 211, 224, 229 |
| abstract_inverted_index.was | 139, 173 |
| abstract_inverted_index.GBA2 | 83, 116 |
| abstract_inverted_index.acid | 137 |
| abstract_inverted_index.bile | 225 |
| abstract_inverted_index.from | 14 |
| abstract_inverted_index.into | 223 |
| abstract_inverted_index.mice | 52, 104 |
| abstract_inverted_index.only | 197 |
| abstract_inverted_index.show | 42 |
| abstract_inverted_index.that | 154, 178, 204, 213 |
| abstract_inverted_index.thus | 227 |
| abstract_inverted_index.type | 5, 89 |
| abstract_inverted_index.well | 142 |
| abstract_inverted_index.with | 123 |
| abstract_inverted_index.(GD), | 32 |
| abstract_inverted_index.(NPC) | 7 |
| abstract_inverted_index.GCase | 74, 97, 109, 125 |
| abstract_inverted_index.LIMP2 | 169, 179, 190, 205, 214 |
| abstract_inverted_index.NPC1, | 59 |
| abstract_inverted_index.laden | 162 |
| abstract_inverted_index.lipid | 161 |
| abstract_inverted_index.liver | 172 |
| abstract_inverted_index.might | 218 |
| abstract_inverted_index.newly | 95 |
| abstract_inverted_index.seems | 194 |
| abstract_inverted_index.their | 73 |
| abstract_inverted_index.these | 192 |
| abstract_inverted_index.which | 16 |
| abstract_inverted_index.(GPNMB | 149 |
| abstract_inverted_index.Livers | 100 |
| abstract_inverted_index.caused | 9 |
| abstract_inverted_index.cells. | 164 |
| abstract_inverted_index.efflux | 13, 57 |
| abstract_inverted_index.export | 220 |
| abstract_inverted_index.facing | 81 |
| abstract_inverted_index.formed | 96 |
| abstract_inverted_index.latter | 156 |
| abstract_inverted_index.levels | 117 |
| abstract_inverted_index.liver, | 129 |
| abstract_inverted_index.liver. | 189 |
| abstract_inverted_index.livers | 50 |
| abstract_inverted_index.recent | 202 |
| abstract_inverted_index.showed | 105, 153, 177 |
| abstract_inverted_index.tended | 118 |
| abstract_inverted_index.(GCase) | 37 |
| abstract_inverted_index.Earlier | 165 |
| abstract_inverted_index.Gaucher | 30 |
| abstract_inverted_index.GlcCer, | 39 |
| abstract_inverted_index.Kupffer | 163 |
| abstract_inverted_index.Similar | 28 |
| abstract_inverted_index.channel | 209 |
| abstract_inverted_index.content | 77 |
| abstract_inverted_index.cytosol | 80 |
| abstract_inverted_index.disease | 3, 31 |
| abstract_inverted_index.enzymes | 134 |
| abstract_inverted_index.harbors | 206 |
| abstract_inverted_index.lacking | 53 |
| abstract_inverted_index.markers | 145, 157 |
| abstract_inverted_index.mediate | 219 |
| abstract_inverted_index.prompts | 210 |
| abstract_inverted_index.protein | 88, 110 |
| abstract_inverted_index.reduced | 108 |
| abstract_inverted_index.status, | 75 |
| abstract_inverted_index.storage | 2 |
| abstract_inverted_index.(LIMP2), | 91 |
| abstract_inverted_index.elevated | 44 |
| abstract_inverted_index.impaired | 11 |
| abstract_inverted_index.increase | 167 |
| abstract_inverted_index.integral | 86 |
| abstract_inverted_index.localize | 198 |
| abstract_inverted_index.membrane | 87 |
| abstract_inverted_index.observed | 140 |
| abstract_inverted_index.patients | 33, 41 |
| abstract_inverted_index.protects | 228 |
| abstract_inverted_index.reported | 166 |
| abstract_inverted_index.(GlcCer). | 27 |
| abstract_inverted_index.activity. | 113 |
| abstract_inverted_index.contrast, | 115 |
| abstract_inverted_index.deficient | 34 |
| abstract_inverted_index.degrading | 38 |
| abstract_inverted_index.enzymatic | 112 |
| abstract_inverted_index.expressed | 159 |
| abstract_inverted_index.increased | 122, 130, 144 |
| abstract_inverted_index.lysosomal | 1, 21, 55, 85, 133 |
| abstract_inverted_index.partially | 107 |
| abstract_inverted_index.patients, | 72 |
| abstract_inverted_index.secondary | 20 |
| abstract_inverted_index.(cathepsin | 135 |
| abstract_inverted_index.biomarkers | 66 |
| abstract_inverted_index.confirmed. | 174 |
| abstract_inverted_index.expression | 63, 131 |
| abstract_inverted_index.lysosomes, | 15 |
| abstract_inverted_index.lysosomes. | 99 |
| abstract_inverted_index.80-week-old | 102 |
| abstract_inverted_index.Npc1−/− | 103, 128, 171, 188, 216 |
| abstract_inverted_index.accompanied | 18 |
| abstract_inverted_index.ceramidase) | 138 |
| abstract_inverted_index.cholesterol | 12, 56, 208, 222 |
| abstract_inverted_index.deficiency. | 126 |
| abstract_inverted_index.established | 65 |
| abstract_inverted_index.hepatocytes | 185, 193, 217 |
| abstract_inverted_index.lipid-laden | 68 |
| abstract_inverted_index.macrophages | 69, 148 |
| abstract_inverted_index.recognition | 203 |
| abstract_inverted_index.speculation | 212 |
| abstract_inverted_index.transporter | 58, 93 |
| abstract_inverted_index.accumulation | 22 |
| abstract_inverted_index.cholesterol. | 48 |
| abstract_inverted_index.galectin-3). | 151 |
| abstract_inverted_index.glucosylated | 47 |
| abstract_inverted_index.hepatocytes. | 230 |
| abstract_inverted_index.investigated | 61 |
| abstract_inverted_index.particularly | 181 |
| abstract_inverted_index.reciprocally | 121 |
| abstract_inverted_index.Unexpectedly, | 175 |
| abstract_inverted_index.overexpressed | 182 |
| abstract_inverted_index.sphingomyelin | 24 |
| abstract_inverted_index.Niemann–Pick | 4 |
| abstract_inverted_index.lipid-stressed | 147 |
| abstract_inverted_index.(endo)lysosomes. | 200 |
| abstract_inverted_index.glucosylceramide | 26 |
| abstract_inverted_index.glucocerebrosidase | 36 |
| abstract_inverted_index.glucosylceramidase | 82 |
| abstract_inverted_index.glucosylsphingosine | 45 |
| abstract_inverted_index.Immunohistochemistry | 152 |
| abstract_inverted_index.immunohistochemistry | 176 |
| cited_by_percentile_year.max | 100 |
| cited_by_percentile_year.min | 90 |
| corresponding_author_ids | https://openalex.org/A5013935159 |
| countries_distinct_count | 2 |
| institutions_distinct_count | 10 |
| corresponding_institution_ids | https://openalex.org/I121797337 |
| citation_normalized_percentile.value | 0.9502936 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | True |