COL6A1, Polyclonal Antibody

Anti -COL6A1, NT (Collagen alpha-1(VI) Chain)

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

Chromosome: 21; NC_000021.8 (47401663..47424963). Location: 21q22.3

Polyclonal

IgG

Rabbit

Recognizes human COL6A1.

Purified
Purified by ammonium sulfate precipitation.

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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 protein COL6A1 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.

Antibodies; Abs to Collagen

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Dilution: ELISA: 1:1,000
Western Blot: 1:50-1:100

108,529 Da[Similar Products]

collagen, type VI, alpha 1

collagen alpha-1(VI) chain; collagen alpha-1(VI) chain; OTTHUMP00000115501; 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]

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, signal peptide; Secreted; Extracellular matrix

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; axon guidance; extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; cell adhesion

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy

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