OBJECTIVE: The physiological and mechanical properties of the skin, the primary tissue affected by @entity785 , depend on the assembly of collagen types I, III and V, which form heterotypic fibers. Collagen V (COLV) regulates heterotypic fiber diameter, and the maintenance of its properties is important for maintaining normal tissue architecture and function. Based on a COLV-induced experimental SSc model, in which overexpression of abnormal COLV was a prominent feature, we assumed that this abnormality could be present in SSc @entity1 and could be correlated to disease duration, skin thickening and disease activity. METHODS: Skin biopsies from 18 @entity1 (6 early-stage and 12 late-stage) and 10 healthy controls were studied. Skin thickening assessment was performed with the @entity272 ( @entity272 ), and activity was calculated using the Valentini Disease Activity Index. Morphology, morphometry of COLV deposition in dermis, as well as, quantitative RT-PCR and 3D-reconstruction of the dermal fibroblast culture were performed. RESULTS: Structurally abnormal COLV was overexpressed in SSc skin, mainly in the early stages of the disease, when compared to normal controls and late-stage. A positive correlation between COLV expression and @entity272 and disease activity was observed. Collagen V alpha-1 and alpha-2 mRNA expression levels were higher in SSc. Tridimensional reconstruction of SSc dermal heterotypic fibers confirmed the presence of atypical COLV. CONCLUSION: Increased synthesis of abnormal COLV and its correlation with disease stage, activity and @entity272 suggest that this collagen can be a possible trigger involved in the pathogenesis of SSc.

Abnormal collagen V deposition in dermis correlates with skin thickening and disease activity in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity272 :: ('MESH:D012871', 'Disease') :: ['Modified Rodnan Skin Score', 'MRSS'] @entity785 :: ('MESH:D012595', 'Disease') :: ['systemic sclerosis']
@entity785 :: (MESH:D012595,Disease) :: ['systemic sclerosis']
