Collagen Type lV, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of COL4 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS106312 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Collagen Type lV (COL4) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing COL4. The ELISA analytical biochemical technique of the MBS106312 kit is based on COL4 antibody-COL4 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect COL4 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, COL4. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

108,529 Da

collagen, type VI, alpha 1

collagen alpha-1(VI) chain; collagen alpha-1(VI) chain; alpha 1 (VI) chain (61 AA); collagen VI, alpha-1 polypeptide

Collagen alpha-1(VI) chain

CO6A1_HUMAN

The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy. [provided by RefSeq, Jul 2008]

COL6A1: Collagen VI acts as a cell-binding protein. Defects in COL6A1 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy. Belongs to the type VI collagen family.

Protein type: Secreted; Extracellular matrix; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; protein complex; membrane; endoplasmic reticulum lumen; lysosomal membrane; extracellular region; collagen type VI; sarcolemma

Molecular Function: platelet-derived growth factor binding

Biological Process: osteoblast differentiation; collagen catabolic process; extracellular matrix disassembly; axon guidance; extracellular matrix organization and biogenesis; cell adhesion

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy

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