We have determined the interactive sites required for dimer formation in type VI collagen. Despite the fact that type VI collagen is a heterotrimer composed of alpha1(VI), alpha2(VI), and alpha3(VI) chains, the formation of dimers is determined principally by interactions of the alpha2(VI) chain. Key components of this interaction are the metal ion-dependent adhesion site (MIDAS) motif of the a...

Adult muscle stem cells, or satellite cells have essential roles in homeostasis and regeneration of skeletal muscles. Satellite cells are located within a niche that includes myofibers and extracellular matrix. The function of specific extracellular matrix molecules in regulating SCs is poorly understood. Here, we show that the extracellular matrix protein collagen VI is a key component of the ...

Previous studies have suggested that procollagen types I and III are the major collagenous gene products of cultured human skin fibroblasts. In this study the expression of 10 different genes, encoding the subunit polypeptides for collagen types I-VI, by human skin fibroblasts in culture was analyzed by molecular hybridizations. Northern transfer analysis demonstrated the presence of specific m...

An mAb was used in conjunction with immunoelectron microscopy to study the ultrastructure and distribution of the type VI collagen network. Type VI collagen in femoral head and costal cartilage was found distributed throughout the matrix but concentrated in areas surrounding chondrocytes. Three-dimensional information gained from high voltage stereo pair electron microscopy showed that the type...

BACKGROUND Overproduction of collagenous fibres types I and III is a common finding of fibrotic injury. Collagen type VI is generally associated with type I. Appearance of fibroblasts expressing alpha-smooth muscle actin (ASMA) and their role in fibrogenesis has been partly defined. However, correlation between renal fibroblasts and accumulation of microfibrillar collagen type VI, as well as it...

We have previously shown that von Willebrand factor (vWF), a glycoprotein which plays a critical role in the adhesion of platelets to injured blood vessels, is present within vascular subendothelium. We investigated the identity of the subendothelial binding site(s) for vWF by examining vWF binding to subendothelial constituents and solubilized a 150-kD protein with SDS-urea that bound vWF. Thi...

The presence or absence of type-VI collagen in mechanoreceptors in the lips and palate of the mouse and gerbil was assessed histochemically. When fresh palatine mucosae and the lips of mice were incubated in 20 mM ATP solution, numerous fibrous long spacing fibers with a periodicity of 100 nm appeared in the interlamellar spaces of Meissner's corpuscles, simple lamellated corpuscles, and the pe...

Patients suffering from collagen VI related myopathies caused by mutations in COL6A1, COL6A2 and COL6A3 often also display skin abnormalities, like formation of keloids or "cigarette paper" scars, dry skin, striae rubrae and keratosis pilaris (follicular keratosis). Here we evaluated if Col6a1 null mice, an established animal model for the muscle changes in collagen VI related myopathies, are a...

Foetal-bovine nuchal ligament and aorta, together with adult-bovine aorta and pregnant uterus, were extracted under dissociative conditions in the absence and in the presence of a reducing agent. A collagenous glycoprotein of Mr 140000 [designated component 140K(VI)], identified in these extracts as the major periodate/Schiff-positive component, was shown to be related to collagen type VI. Dige...

Type VI collagen is a subendothelial constituent that binds von Willebrand factor (vWF) and platelets. The interaction of platelets with type VI collagen and the roles of platelet glycoprotein (GP) receptors and vWF were studied under flow conditions using epi-fluorescent videomicroscopy coupled with digital image processing. We found that surface coverage was less than 6% on collagen VI at a r...