Por favor, use este identificador para citar o enlazar a este item:
http://hdl.handle.net/10261/264060
COMPARTIR / EXPORTAR:
SHARE CORE BASE | |
Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL | DATACITE | |
Título: | Complement factor D (adipsin) levels are elevated in acquired partial lipodystrophy (Barraquer-Simons syndrome) |
Autor: | Corvillo, Fernando; González-Sánchez, Laura CSIC ORCID; López-Lera, Alberto; Arjona, Emilia CSIC ORCID ; Ceccarini, Giovanni; Santini, Ferruccio; Araújo-Vilar, David; Brown, Rebecca J.; Villarroya, Joan; Villarroya, Francesc; Rodríguez de Córdoba, Santiago ; Caballero-Velázquez, Teresa CSIC ORCID; Nozal, Pilar; López-Trascasa, Margarita | Palabras clave: | Adipsin Complement factor D Acquired partial lipodystrophy Barraquer–Simons syndrome Complement system |
Fecha de publicación: | 21-jun-2021 | Editor: | Multidisciplinary Digital Publishing Institute | Citación: | International Journal of Molecular Sciences 22 (12) 6608 (2021) | Resumen: | 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. | Descripción: | 14 p.-7 fig.-2 tab. | Versión del editor: | https://doi.org/10.3390/ijms22126608 | URI: | http://hdl.handle.net/10261/264060 | DOI: | 10.3390/ijms22126608 | ISSN: | 1661-6596 | E-ISSN: | 1422-0067 |
Aparece en las colecciones: | (CIB) Artículos |
Ficheros en este ítem:
Fichero | Descripción | Tamaño | Formato | |
---|---|---|---|---|
ijms_corvillo_2021.pdf | Artículo principal | 23,16 MB | Adobe PDF | Visualizar/Abrir |
CORE Recommender
PubMed Central
Citations
4
checked on 23-abr-2024
SCOPUSTM
Citations
6
checked on 23-abr-2024
WEB OF SCIENCETM
Citations
7
checked on 28-feb-2024
Page view(s)
39
checked on 27-abr-2024
Download(s)
45
checked on 27-abr-2024