نتایج جستجو برای: collagen type vi
تعداد نتایج: 1418081 فیلتر نتایج به سال:
BACKGROUND Among the several challenges faced by bloodsucking arthropods, the vertebrate hemostatic response against blood loss represents an important barrier to efficient blood feeding. Here we report the first inhibitor of collagen-induced platelet aggregation derived from the salivary glands of a black fly (Simulium nigrimanum), named Simplagrin. METHODS AND FINDINGS Simplagrin was expres...
The Bethlem myopathy, a childhood onset autosomal dominant myopathy with joint contractures, has recently been localized to 21q in a series of Dutch families and the alpha 1 and alpha 2 subunits of type VI collagen (COL6A1 and COL6A2) have been postulated as candidate genes. We investigate a large family of French Canadian descent (family 1489) in which the Bethlem myopathy is segregating. Fami...
Congenital muscular dystrophy type Ullrich (UCMD) is a severe disorder of early childhood onset for which currently there is no effective treatment. UCMD commonly is caused by dominant-negative mutations in the genes coding for collagen type VI, a major microfibrillar component of the extracellular matrix surrounding the muscle fibers. To explore RNA interference (RNAi) as a potential therapy f...
objective(s):skin extracellular matrix, which contains type i and type iii collagens, is involved in skin development. the aim of this study was to investigate type iii collagen distribution pattern as well as its changes during pre and post-natal skin morphogenesis in rats. materials and methods: ventral skins of wistar rat embryos at different stages from 10 to 20 gestational day (e10-e20) an...
Type VI collagen (COL6), an extracellular matrix protein, is important in maintaining the integrity of lung tissue. An increase in COL6 mRNA and protein deposition was found in the lungs of patients with pulmonary fibrosis, a chronic inflammatory condition with a strong association with lung cancer. In the present study, we demonstrated overexpression of COL6 in the lungs of non-small cell lung...
Increasing tensile properties and collagen content is a recognized need in articular cartilage tissue engineering. This study tested the hypothesis that multiple applications of chondroitinase ABC (C-ABC), a glycosaminoglycan (GAG) degrading enzyme, could increase construct tensile properties in a scaffold-less approach for articular cartilage tissue engineering. Developing constructs were trea...
Mutations in the three collagen VI genes COL6A1, COL6A2 and COL6A3 cause Bethlem myopathy and Ullrich congenital muscular dystrophy (UCMD). UCMD, a severe disorder characterized by congenital muscle weakness, proximal joint contractures and marked distal joint hyperextensibility, has been considered a recessive condition, and homozygous or compound heterozygous mutations have been defined in CO...
PURPOSE To examine ultrastructurally the composition of extracellular matrix (ECM) materials, the distribution of myocilin, and the colocalization of myocilin with ECM components in the juxtacanalicular tissue (JCT) of normal human eyes. METHODS Postembedding immunoelectron microscopic studies were performed with antibodies specific for major ECM components, including fibronectin, laminin, vi...
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