RT Journal Article
T1 Autoantibodies Against Perilipin 1 as a Cause of Acquired Generalized Lipodystrophy
A1 Corvillo, Fernando
A1 Aparicio, Verónica
A1 López Lera, Alberto
A1 Garrido, Sofía
A1 Araujo-Vilar, David
A1 Miguel González, María P.
A1 López Trascasa, Margarita
K1 Generalized lipodystrophy
K1 Perilipin
K1 Autoimmunity
K1 Lipolysis
K1 Lawrence syndrome
AB Acquired generalized lipodystrophy (AGL) is a rare condition characterized by an altered distribution of adipose tissue and predisposition to develop hepatic steatosis and fibrosis, diabetes, and hypertriglyceridemia. Diagnosis of AGL is based on the observation of generalized fat loss, autoimmunity and lack of family history of lipodystrophy. The pathogenic mechanism of fat destruction remains unknown but evidences suggest an autoimmune origin. Anti-adipocyte antibodies have been previously reported in patients with AGL, although their involvement in the pathogenesis has been poorly studied and the autoantibody target/s remain/s to be identified. Using a combination of immunochemical and cellular studies, we investigated the presence of anti-adipocyte autoantibodies in patients with AGL, acquired partial lipodystrophy, localized lipoatrophy due to intradermic insulin injections or systemic lupus erythematosus. Moreover, the impact of anti-adipocyte autoantibodies from AGL patients was assessed in cultured mouse preadipocytes. Following this approach, we identified anti-perilipin 1 IgG autoantibodies in the serum of patients with autoimmune variety-AGL, but in no other lipodystrophies tested. These autoantibodies altered the ability of perilipin 1 to regulate lipolysis in cultured preadipocytes causing abnormal, significantly elevated basal lipolysis. Our data provide strong support for the conclusion that perilipin 1 autoantibodies are a cause of generalized lipodystrophy in these patients
PB Frontiers Media
SN 1664-3224
YR 2018
FD 2018
LK http://hdl.handle.net/10347/22230
UL http://hdl.handle.net/10347/22230
LA eng
NO Corvillo F, Aparicio V, López-Lera A, Garrido S, Araújo-Vilar D, de Miguel MP and López-Trascasa M (2018) Autoantibodies Against Perilipin 1 as a Cause of Acquired Generalized Lipodystrophy. Front. Immunol. 9:2142
NO FC, ML-T, AL-L, and SG were supported by grants PI15-00255 from Instituto de Salud Carlos III (ISCIII, Ministerio de Economía y Competitividad) and Fondos FEDER, Complemento II-CM network (B2017/BMD3673), Acciones Cooperativas y Complementarias Intramurales (ACCI) from CIBERER (ISCIII), and Fundación SENEFRO. MdM was supported by Roche Farma SA and Foundation Domingo Martínez. VA was financed by Consejería de Educación, Juventud y Deporte of Comunidad de Madrid and by Fondo Social Europeo (Programa Operativo de Empleo Juvenil, and Iniciativa de Empleo Juvenil (YEI), (PEJ15/BIO/AI/0045). DA-V was supported by the intramural research program of the Xunta de Galicia (Programa de Consolidación e Estructuración de Unidades de Investigación Competitivas, grant ED341b 2017/19), by the Instituto de Salud Carlos III (grant number: PI08-1449) and the European Regional Development Fund, FEDER and by the Asociación Española de Familiares y Afectados de Lipodistrofias (AELIP)
DS Minerva
RD 24 abr 2026