Fibrogenesis failure of type V collagen observed in pulmonary and cutaneous fibroblast culture reinforce the pathogenic participation of this collagen in the pathway of systemic sclerosis

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author TEODORO, W. R. FMUSP-HC
MORAIS, J. FMUSP-HC
MARTIN, P. FMUSP-HC
VELOSA, A. P. P. FMUSP-HC
CARRASCO, S. FMUSP-HC
SOUZA, R. B. C.
KATAYAMA, M. L. FMUSP-HC
GOLDEINSTEIN-SCHAINBERG, C. FMUSP-HC
PARRA, E. R. FMUSP-HC
CAPELOZZI, V. L. FMUSP-HC
YOSHINARI, N. H. FMUSP-HC
dc.date.issued 2012
dc.identifier.citation HISTOPATHOLOGY, v.61, suppl.1, Special Issue, p.164-165, 2012
dc.identifier.issn 0309-0167
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/2991
dc.description.abstract Introduction: Unusual type V collagen (COLV) accumulation was demonstrated in systemic sclerosis (SSc) by our group. In this regard, this study analyzed tridimensional reconstruction (3D), biochemical and molecular profile of COLVα1 and COLVα2 chains in pulmonary and cutaneous fibroblasts culture from patients with SSc. Materials and Methods: Pulmonary and cutaneous fibroblasts for culture were obtained from 7 patients with SSc and from six controls respectively. COLV 3D reconstruction was performed by confocal microscopy. COLVα1 and COLVα2 gene expression was performed by RT-PCR and COLV protein expression by immunoblotting. Results: COL V 3D reconstruction showed distorted and strongly thickened fibers with irregular bundles resulting in a dense network in lung and skin fibroblast cultures from SSc patients compared to the thin fibers from fibroblast controls. Collagen quantification showed significant increased COLV fiber expression in SSc cutaneous and pulmonary fibroblasts (P<0.01) compared with the respective controls. In the same way, molecular evaluation demonstrated an increased significance (P=0.05) of COLVα1 and COLVα2 mRNA expression in cutaneous and pulmonary fibroblasts from SSc patients to that of control groups. The immunoblotting analysis demonstrated the increased weight of the molecular COLV chains. Conclusion: COLV overexpression and an unusual organization of these fibers including molecular and biochemical changes, suggest an interference process of the COLV fibrillogenesis in patients with SSc, reinforcing the participation of this collagen in SSc pathogenesis and open new therapeutic perspectives for these patients.
dc.language.iso eng
dc.publisher WILEY-BLACKWELL
dc.relation.ispartof Histopathology
dc.rights restrictedAccess
dc.title Fibrogenesis failure of type V collagen observed in pulmonary and cutaneous fibroblast culture reinforce the pathogenic participation of this collagen in the pathway of systemic sclerosis
dc.type conferenceObject
dc.rights.holder Copyright WILEY-BLACKWELL
dc.description.conferencedate SEP 30-OCT 05, 2012
dc.description.conferencelocal Cape Town, SOUTH AFRICA
dc.description.conferencename 29th Congress of the International-Academy-of-Pathology
dc.description.group LIM/17
dc.description.group LIM/24
dc.type.category meeting abstract
dc.type.version publishedVersion
hcfmusp.author TEODORO, W. R.:FM:MCM
hcfmusp.author MORAIS, J.:FM:
hcfmusp.author MARTIN, P.:FM:
hcfmusp.author VELOSA, A. P. P.:HC:LIM/17
hcfmusp.author CARRASCO, S.:FM:
hcfmusp.author KATAYAMA, M. L.:FM:MDR
hcfmusp.author GOLDEINSTEIN-SCHAINBERG, C.:HC:LIM/17
hcfmusp.author PARRA, E. R.:FM:
hcfmusp.author CAPELOZZI, V. L.:FM:MPT
hcfmusp.author YOSHINARI, N. H.:FM:MCM
hcfmusp.author.external · SOUZA, R. B. C.:Univ Sao Paulo, Div Rheumatol, Sao Paulo, Brazil
hcfmusp.origem.id WOS:000308126900472
hcfmusp.publisher.city HOBOKEN
hcfmusp.publisher.country USA
dc.description.index MEDLINE
hcfmusp.citation.wos 0
hcfmusp.affiliation.country Brasil


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