RT Journal Article SR Electronic T1 Up-regulation of collagen type V mRNA in a model of systemic sclerosis JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP p3803 VO 38 IS Suppl 55 A1 Roberta Gonçalves Marangoni A1 Ana Paula P. Velosa A1 Edwin E. Parra A1 Jymenez de Moraes A1 Vera L. Capelozzi A1 Walcy R. Teodoro A1 Natalino H. Yoshinari YR 2011 UL http://erj.ersjournals.com/content/38/Suppl_55/p3803.abstract AB Background: We have described an animal model of systemic sclerosis (SSc) induced by type V collagen (COLV) that resembles the human disease. The aim of this study was to investigate the early disease in the lung of this model with special emphasis on collagen deposition and mRNA collagen synthesis.Methods: Female rabbits from New Zealand lineage were immunized with COLV plus Freund's adjuvant (FA). Animals immunized only with FA were used as controls. The animals were sacrificed at day-7, day-75 and day-210; and the lungs submitted to immunofluorescence, real-time qPCR and biochemical examination.Results: The immunolabeling for the COL I, III and V by immunofluorescence showed an intense expression of COLV in the bronchovascular interstitium near the inflammatory infiltrate of COLV-rabbits at day-210. Additionally, morphologic analysis demonstrated that the progressive remodeling of the extracellular matrix observed in this group of animals was characterized by thickened COLV deposition with distorted fibrils. In accordance with these observations, the real-time qPCR revealed markedly up-regulation of COLV mRNA of the COLV-group at day-210 comparing with controls (p<0.001). The content of hydroxyproline was compared for COLV-group and controls at day-7, day-75 and day-210, suggesting that the day-210 group represents an early disease without established fibrosis.Conclusions: We found an early up-regulation of COLV mRNA in the lung tissue of this experimental model emphasizing the role of this protein in the pathogenesis of SSc. In addition, our results reinforce the importance of this model, emerging promising to study the early stages of pulmonary fibrosis in this severe disease.