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Ultrastructural study and molecular fibrillogenesis of COL V and its chains, relation with SPARC protein gene and impact of genic silence in skin and lung biopsies of patients with systemic sclerosis


Fibrosis is the main feature of the autoimmune disease referred to as systemic sclerosis or scleroderma (SSc). There are currently, most authors justifies the skin and lung remodeling in these patients to intense synthesis of COLI and COLIII during the progression of ES. But recent studies carried out from our group show that in the early stages of the disease, the skin and lungs of the patients present a distorted fibrillar pattern, increased COLV synthesis and COL(V)alpha2 gene expression. These parameters were related with disease activity. Such results corroborates with earlier studies in experimental SSc by immunizing healthy rabbits with COLV, which we observed the vascular reactivity, autoimmunity and fibrosis in the skin and internal organs, similar to the disease in humans. Considering that the cutaneous and pulmonary affection present in SSc, may to be related to COLV fibrillogenesis and /or its chains, as well as the expression of the protein SPARC, a matricelular protein important in the fibrosis formation, our proposal is to study the COLV and COLV chains fibrillogenesis on ultrastructural and molecular bases, their relationship with the expression of SPARC protein and COLI and COLIII, as well as the impact of the COL(V)alpha2 chain gene silencing, using surgical specimens from skin and lung of patients with SSc . So if proven that the COL(V)alpha2 chain interferes in the formation of heterotypic fibers and consequently modifying the extracellular matrix in these patients, it may have an important role in fibrosis, vascular reactivity and autoimmunity present at the SSc. (AU)

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