Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome) Article Swipe
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· 2021
· Open Access
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· DOI: https://doi.org/10.3390/ijms22126608
Complement overactivation has been reported in most patients with Barraquer–Simons syndrome (BSS), a rare form of acquired partial lipodystrophy. Complement Factor D (FD) is a serine protease with a crucial role in the activation of the alternative pathway of the complement system, which is mainly synthesized by adipose tissue. However, its role in the pathogenesis of BSS has not been addressed. In this study, plasma FD concentration was measured in 13 patients with BSS, 20 patients with acquired generalized lipodystrophy, 22 patients with C3 glomerulopathy (C3G), and 50 healthy controls. Gene expression and immunohistochemistry studies were assayed using atrophied adipose tissue from a patient with BSS. We found significantly elevated FD levels in BSS cases compared with the remaining cohorts (p < 0.001). There were no significant differences in FD levels between sexes but FD was strongly and directly associated with age in BSS (r = 0.7593, p = 0.0036). A positive correlation between FD and C3 was seen in patients with C3G, characterized by decreased FD levels due to chronic C3 consumption, but no correlation was detected for BSS. Following mRNA quantification in the patient’s adipose tissue, we observed decreased CFD and C3 but elevated C5 transcript levels. In contrast, the increased FD staining detected in the atrophied areas reflects the effects of persistent tissue damage on the adipose tissue, thus providing information on the ongoing pathogenic process. Our results suggest that FD could be a reliable diagnostic biomarker involved in the pathophysiology of BSS by promoting unrestrained local complement system activation in the adipose tissue environment.
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- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3390/ijms22126608
- https://www.mdpi.com/1422-0067/22/12/6608/pdf?version=1624249981
- OA Status
- gold
- Cited By
- 15
- References
- 49
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W3175765750
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- OpenAlex ID
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https://openalex.org/W3175765750Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.3390/ijms22126608Digital Object Identifier
- Title
-
Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome)Work title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2021Year of publication
- Publication date
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2021-06-21Full publication date if available
- Authors
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Fernando Corvillo, Laura González-Sánchez, Alberto López‐Lera, Emilia Arjona, Giovanni Ceccarini, Ferruccio Santini, David Araújo‐Vilar, Rebecca J. Brown, Joan Villarroya, Francesc Villarroya, Santiago Rodrı́guez de Córdoba, Teresa Caballero, Pilar Nozal, Margarita López‐TrascasaList of authors in order
- Landing page
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https://doi.org/10.3390/ijms22126608Publisher landing page
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https://www.mdpi.com/1422-0067/22/12/6608/pdf?version=1624249981Direct link to full text PDF
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
-
https://www.mdpi.com/1422-0067/22/12/6608/pdf?version=1624249981Direct OA link when available
- Concepts
-
Adipose tissue, Lipodystrophy, Complement system, Internal medicine, Endocrinology, Biomarker, Pathogenesis, Complement factor I, Medicine, Biology, Pathology, Immunology, Antibody, Biochemistry, Antiretroviral therapy, Viral load, Human immunodeficiency virus (HIV)Top concepts (fields/topics) attached by OpenAlex
- Cited by
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15Total citation count in OpenAlex
- Citations by year (recent)
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2025: 6, 2024: 1, 2023: 5, 2022: 1, 2021: 2Per-year citation counts (last 5 years)
- References (count)
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49Number of works referenced by this work
- Related works (count)
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10Other works algorithmically related by OpenAlex
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| abstract_inverted_index.adipose | 47, 99, 186, 219, 255 |
| abstract_inverted_index.assayed | 96 |
| abstract_inverted_index.between | 131, 153 |
| abstract_inverted_index.chronic | 170 |
| abstract_inverted_index.cohorts | 119 |
| abstract_inverted_index.crucial | 29 |
| abstract_inverted_index.effects | 212 |
| abstract_inverted_index.healthy | 88 |
| abstract_inverted_index.levels. | 198 |
| abstract_inverted_index.ongoing | 226 |
| abstract_inverted_index.partial | 17 |
| abstract_inverted_index.pathway | 37 |
| abstract_inverted_index.patient | 103 |
| abstract_inverted_index.results | 230 |
| abstract_inverted_index.studies | 94 |
| abstract_inverted_index.suggest | 231 |
| abstract_inverted_index.system, | 41 |
| abstract_inverted_index.tissue, | 187, 220 |
| abstract_inverted_index.tissue. | 48 |
| abstract_inverted_index.0.0036). | 149 |
| abstract_inverted_index.However, | 49 |
| abstract_inverted_index.acquired | 16, 77 |
| abstract_inverted_index.compared | 115 |
| abstract_inverted_index.detected | 177, 205 |
| abstract_inverted_index.directly | 138 |
| abstract_inverted_index.elevated | 109, 195 |
| abstract_inverted_index.involved | 240 |
| abstract_inverted_index.measured | 68 |
| abstract_inverted_index.observed | 189 |
| abstract_inverted_index.patients | 7, 71, 75, 81, 160 |
| abstract_inverted_index.positive | 151 |
| abstract_inverted_index.process. | 228 |
| abstract_inverted_index.protease | 26 |
| abstract_inverted_index.reflects | 210 |
| abstract_inverted_index.reliable | 237 |
| abstract_inverted_index.reported | 4 |
| abstract_inverted_index.staining | 204 |
| abstract_inverted_index.strongly | 136 |
| abstract_inverted_index.syndrome | 10 |
| abstract_inverted_index.Following | 180 |
| abstract_inverted_index.atrophied | 98, 208 |
| abstract_inverted_index.biomarker | 239 |
| abstract_inverted_index.contrast, | 200 |
| abstract_inverted_index.controls. | 89 |
| abstract_inverted_index.decreased | 165, 190 |
| abstract_inverted_index.increased | 202 |
| abstract_inverted_index.promoting | 247 |
| abstract_inverted_index.providing | 222 |
| abstract_inverted_index.remaining | 118 |
| abstract_inverted_index.Complement | 0, 19 |
| abstract_inverted_index.activation | 33, 252 |
| abstract_inverted_index.addressed. | 60 |
| abstract_inverted_index.associated | 139 |
| abstract_inverted_index.complement | 40, 250 |
| abstract_inverted_index.diagnostic | 238 |
| abstract_inverted_index.expression | 91 |
| abstract_inverted_index.pathogenic | 227 |
| abstract_inverted_index.persistent | 214 |
| abstract_inverted_index.transcript | 197 |
| abstract_inverted_index.alternative | 36 |
| abstract_inverted_index.correlation | 152, 175 |
| abstract_inverted_index.differences | 127 |
| abstract_inverted_index.generalized | 78 |
| abstract_inverted_index.information | 223 |
| abstract_inverted_index.patient’s | 185 |
| abstract_inverted_index.significant | 126 |
| abstract_inverted_index.synthesized | 45 |
| abstract_inverted_index.consumption, | 172 |
| abstract_inverted_index.environment. | 257 |
| abstract_inverted_index.pathogenesis | 54 |
| abstract_inverted_index.unrestrained | 248 |
| abstract_inverted_index.characterized | 163 |
| abstract_inverted_index.concentration | 66 |
| abstract_inverted_index.significantly | 108 |
| abstract_inverted_index.glomerulopathy | 84 |
| abstract_inverted_index.lipodystrophy, | 79 |
| abstract_inverted_index.lipodystrophy. | 18 |
| abstract_inverted_index.overactivation | 1 |
| abstract_inverted_index.quantification | 182 |
| abstract_inverted_index.pathophysiology | 243 |
| abstract_inverted_index.Barraquer–Simons | 9 |
| abstract_inverted_index.immunohistochemistry | 93 |
| cited_by_percentile_year.max | 99 |
| cited_by_percentile_year.min | 89 |
| corresponding_author_ids | https://openalex.org/A5000094605 |
| countries_distinct_count | 3 |
| institutions_distinct_count | 14 |
| corresponding_institution_ids | https://openalex.org/I4210103477, https://openalex.org/I4210111366 |
| sustainable_development_goals[0].id | https://metadata.un.org/sdg/3 |
| sustainable_development_goals[0].score | 0.7900000214576721 |
| sustainable_development_goals[0].display_name | Good health and well-being |
| citation_normalized_percentile.value | 0.70805296 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | False |