OPLL antibody; COL6A1 antibody; "collagen VI; alpha-1 polypeptide antibody"; alpha 1 (VI) chain (61 AA) antibody; collagen alpha-1(VI) chain antibody; "collagen; type VI; alpha 1 antibody"

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total COL6A1 protein.

Antibodies were purified by antigen-affinity chromatography.

Rabbit IgG in 0.1M Tris, 0.1M Glycine, 20% Glycerol (pH7). 0.01% Thimerosal was added as a preservative.

1 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-COL6A1 antibody 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.

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]

Total protein Ab

Western Blot (WB)

Western blotting: 1:500-1:3000

Sample (30 ug of whole cell lysate) A: BCL-1 5% SDS PAGE diluted at 1:500

108,529 Da

collagen, type VI, alpha 1

collagen alpha-1(VI) chain

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; Secreted, signal peptide; Extracellular matrix

Chromosomal Location of Human Ortholog: 21q22.3

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

Molecular Function: platelet-derived growth factor binding

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

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy

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