COLLAGEN V AND DECORIN INTERACTION ARE INVOLVED IN PULMONARY FIBROSIS OF SSc
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Tipo de produção
conferenceObject
Data de publicação
2012
Título da Revista
ISSN da Revista
Título do Volume
Editora
OXFORD UNIV PRESS
Autores
MARCELINO, A.
CRUZ, I. C. Brindo da
Citação
RHEUMATOLOGY, v.51, suppl.2, p.30-30, 2012
Resumo
Aims. Type V collagen (COL V) is involved in SSc pathogenesis since immunization of health rabbits with this protein induces an experimental model reproducing the main pathogenic manifestation of this disease. We have demonstrated an increased amount of unusual COL V fibrils deposition in lung of SSc patients indicating an important role for this protein in fibrosis. Formation of fibrotic tissue can be induced both by cytokines and cell–matrix interaction involving signalization mechanism. COL V and decorin participate of this mechanism interfering with fibrilogenesis. The aim was to evaluate COL V and decorin expression in pulmonary tissue and to characterize biochemical profile of COLV from lung fibroblasts culture from SSc patients. Methods. We evaluated COL V and decorin expression as well as 3D reconstruction using IF in lung specimens from six patients with SSc without pulmonary hypertension and six normal individuals died from trauma. The amount of COL V in lung sections was evaluated with software Image Pro-Plus 6.0 in Olympus-BX51. Quantitative immunoblotting was used to characterize COL V biochemistry from lung fibroblasts culture. Results. It was found that the structure of COL V fibres was distorted and strongly thickened in lung tissue from SSc patients compared with thin fibres pattern in the healthy controls. Decorin was distributed around COL V fibrils in the bronchovascular interstitium and vascular walls. Histomorphometric analysis of SSc lung demonstrated increased expression of both COL V and decorin when compared with the control (68.52 + 7.36% vs 5.01 + 2.12%, P < 0.01 and 22.99 + 0.59% vs 32.93 + 3.81%, P = 0.01, respectively). The semiquantitative immunoblotting detected an increased high molecular weight COL V fraction in patients when compared with the controls (P = 0.02). Conclusions. The overexpression and unusual organization of COL V fibres with biochemical changes associated to increased decorin indicates that matrix signalization pathway is involved in COL V fibrillogenesis process in SSc pulmonary fibrosis.