نتایج جستجو برای: collagen type vi
تعداد نتایج: 1418081 فیلتر نتایج به سال:
von Willebrand factor (VWF) plays an important role in coagulation as a carrier protein for factor VIII (FVIII) and in primary hemostasis with binding of VWF to exposed subendothelial collagens. The most hemostatically important forms of collagen include the fibrillar collagens I and III, and the microfibrillar collagen VI. VWF binds to collagen I and III via theVWFA1andA3domainswhereas only th...
Connective tissue microfibrils are a group of unrelated, thin fdamentous structural macromolecules which have historically been loosely linked together on the basis of their gross morphological characteristics (1.2). The primary biological purpose of such structures apparently lies in the provision of connecting links between the major elements of connective tissues, cells and the basal lamina,...
Congenital muscular dystrophies with collagen VI deficiency are inherited muscle disorders with a broad spectrum of clinical presentation and are caused by mutations in one of COL6A1-3 genes. Muscle pathology is characterized by fiber size variation and increased interstitial fibrosis and adipogenesis. In this study, we define critical events that contribute to muscle weakness and fibrosis in a...
The functional importance of platelet endothelial cell adhesion molecule-1 (PECAM-1/CD31) in platelets is unclear. Because PECAM-1 represents a newly assigned immunoglobulin-ITIM superfamily member expressed on the surface of platelets, it was hypothesized that it may play an important regulatory role in modulating ITAM-bearing receptors such as collagen (GP)VI receptor and FcgammaRIIA. To exam...
BACKGROUND AND PURPOSE Mutations in collagen VI-related genes (COL6A1, COL6A2, and COL6A3) cause Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). These were previously believed to be separate disease entities, but they are now both classified as collagen VI-related myopathies, which cover a broad clinical spectrum. We aimed to analyze the clinical, pathologic, and genetic...
Carcinoembryonic antigen cell adhesion molecule-1 (CEACAM1) is a surface glycoprotein expressed on various blood cells, epithelialcells,andvascularcells.CEACAM1 possesses adhesive and signaling properties mediated by its intrinsic immunoreceptor tyrosine-based inhibitory motifs that recruit SHP-1 protein-tyrosine phosphatase. In this study, we demonstrate that CEACAM1 is expressed on the surfac...
Certain strains of Streptococcus sanguis adhere selectively to human platelets (Adh+) and, in plasma, induce them to aggregate into in vitro thrombi (Agg+). The induction of aggregation is mediated by the platelet aggregation-associated protein (PAAP) expressed on the cell surface of the streptococcus. In endocarditis, expression of PAAP may be regulated by association with host proteins on dam...
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