Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases Article Swipe
Maria J. Ferraz
,
André R. A. Marques
,
Monique D. Appelman
,
Marri Verhoek
,
Anneke Strijland
,
Mina Mirzaian
,
Saskia Scheij
,
Cécile Ouairy
,
D. Lahav
,
Patrick Wisse
,
Herman S. Overkleeft
,
Rolf G. Boot
,
Johannes M. F. G. Aerts
·
YOU?
·
· 2016
· Open Access
·
· DOI: https://doi.org/10.1002/1873-3468.12104
YOU?
·
· 2016
· Open Access
·
· DOI: https://doi.org/10.1002/1873-3468.12104
Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity of glycosphingolipid catabolizing glycosidases. We investigated the molecular basis of the formation of glucosylsphingosine and globotriaosylsphingosine during deficiency of glucocerebrosidase (Gaucher disease) and α‐galactosidase A (Fabry disease). Independent genetic and pharmacological evidence is presented pointing to an active role of acid ceramidase in both processes through deacylation of lysosomal glycosphingolipids. The potential pathophysiological relevance of elevated glycosphingoid bases generated through this alternative metabolism in patients suffering from lysosomal glycosidase defects is discussed.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1002/1873-3468.12104
- https://febs.onlinelibrary.wiley.com/doi/pdfdirect/10.1002/1873-3468.12104
- OA Status
- bronze
- Cited By
- 135
- References
- 45
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W2329337854
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
-
https://openalex.org/W2329337854Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.1002/1873-3468.12104Digital Object Identifier
- Title
-
Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidasesWork title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2016Year of publication
- Publication date
-
2016-02-22Full publication date if available
- Authors
-
Maria J. Ferraz, André R. A. Marques, Monique D. Appelman, Marri Verhoek, Anneke Strijland, Mina Mirzaian, Saskia Scheij, Cécile Ouairy, D. Lahav, Patrick Wisse, Herman S. Overkleeft, Rolf G. Boot, Johannes M. F. G. AertsList of authors in order
- Landing page
-
https://doi.org/10.1002/1873-3468.12104Publisher landing page
- PDF URL
-
https://febs.onlinelibrary.wiley.com/doi/pdfdirect/10.1002/1873-3468.12104Direct link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
bronzeOpen access status per OpenAlex
- OA URL
-
https://febs.onlinelibrary.wiley.com/doi/pdfdirect/10.1002/1873-3468.12104Direct OA link when available
- Concepts
-
Glycosphingolipid, Glucocerebroside, Catabolism, Glucocerebrosidase, Biochemistry, Sphingolipid, Chemistry, Gaucher's disease, Enzyme, Disease, Internal medicine, MedicineTop concepts (fields/topics) attached by OpenAlex
- Cited by
-
135Total citation count in OpenAlex
- Citations by year (recent)
-
2025: 17, 2024: 12, 2023: 14, 2022: 9, 2021: 17Per-year citation counts (last 5 years)
- References (count)
-
45Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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| corresponding_author_ids | https://openalex.org/A5013935159 |
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| corresponding_institution_ids | https://openalex.org/I121797337, https://openalex.org/I2802928900 |
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