Fibroblast abnormalities in the pathogenesis of systemic sclerosis [artículo]
Por: Rey Cerros, Manuel José del [Instituto de Investigación i+12]
| Usátegui Corral, Alicia [Instituto de Investigación i+12] | Pablos Álvarez, José Luis [Reumatología].
Colaborador(es): Instituto de Investigación imas12.
Editor: Expert Review of Clinical Immunology, 2011Descripción: 7(4):491-498.Recursos en línea: Solicitar documento Resumen: Systemic sclerosis (SSc) is a chronic systemic disease characterized by autoimmunity, vascular lesions and progressive fibrosis. The fibrotic component is dominant in SSc compared with other vascular or autoimmune diseases and determines its prognosis and therapeutic refractoriness. Fibroblasts are responsible for abnormal extracellular matrix accumulation. Studies in cultured SSc skin fibroblasts have facilitated the identification of potential pathways involved in their profibrotic phenotype. Profibrotic fibroblasts characterized by abnormal growth and extracellular matrix synthesis may differentiate or expand from normal resident fibroblasts. Recruitment of bone marrow-derived progenitors and transdifferentiation of different cell lineages might also be involved. Multiple factors and signaling pathways appear to be involved in the development or persistence of the SSc fibroblast phenotype. Although their relative relevance and interplay are unclear, aberrant TGF-beta signaling seems pivotal and constitutes the best characterized therapeutic target.
| Tipo de ítem | Ubicación actual | Signatura | Estado | Fecha de vencimiento |
|---|---|---|---|---|
Artículo
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PC3430 (Navegar estantería) | Disponible |
Navegando Hospital Universitario 12 de Octubre Estantes Cerrar el navegador de estanterías
Formato Vancouver:
Usategui A, del Rey MJ, Pablos JL. Fibroblast abnormalities in the
pathogenesis of systemic sclerosis. Expert Rev Clin Immunol. 2011;7(4):491-8.
PMID: 21790292
Contiene 105 referencias
Systemic sclerosis (SSc) is a chronic systemic disease characterized by autoimmunity, vascular lesions and progressive fibrosis. The fibrotic component is dominant in SSc compared with other vascular or autoimmune diseases and determines its prognosis and therapeutic refractoriness. Fibroblasts are responsible for abnormal extracellular matrix accumulation. Studies in cultured SSc skin fibroblasts have facilitated the identification of potential pathways involved in their profibrotic phenotype. Profibrotic fibroblasts characterized by abnormal growth and extracellular matrix synthesis may differentiate or expand from normal resident fibroblasts. Recruitment of bone marrow-derived progenitors and transdifferentiation of different cell lineages might also be involved. Multiple factors and signaling pathways appear to be involved in the development or persistence of the SSc fibroblast phenotype. Although their relative relevance and interplay are unclear, aberrant TGF-beta signaling seems pivotal and constitutes the best characterized therapeutic target.
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