COL6A2 / Collagen VI, Monoclonal Antibody

Anti-COL6A2 / Collagen VI Antibody (clone 2C5-F2) IHC-plus; COL6A2; Collagen alpha-2(VI) chain; Collagen; type VI; alpha 2; Collagen alpha 2(vi); PP3610; Collagen VI; Human COL6A2; Collagen VI

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

Monoclonal

IgG2a,k

2C5-F2

Mouse

Human COL6A2 / Collagen VI

Protein A Purified

PBS, pH 7.4.

0.25 mg/ml (lot specific)

Store at -20 degree C or lower. Aliquot to avoid repeated freezing and thawing.

Small volumes of anti-COL6A2 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.

Family: Collagen

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA, IHC-P (10 ug/ml), WB
Usage: Sandwich ELISA: Recombinant protein. Western Blot: Transfected lysate, Recombinant protein. Immunohistochemistry: Paraffin.

Anti-COL6A2 / Collagen VI antibody IHC staining of human spleen. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 10 ug/ml.

Western Blot analysis of COL6A2 expression in transfected 293T cell line by COL6A2 monoclonal antibody (M01), clone 2C5-F2.Lane 1: COL6A2 transfected lysate(109 KDa).Lane 2: Non-transfected lysate.

87,280 Da

collagen, type VI, alpha 2

collagen alpha-2(VI) chain; collagen alpha-2(VI) chain; collagen VI, alpha-2 polypeptide; human mRNA for collagen VI alpha-2 C-terminal globular domain

Collagen alpha-2(VI) chain

CO6A2_HUMAN

This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq, Jul 2008]

COL6A2: Collagen VI acts as a cell-binding protein. Defects in COL6A2 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 COL6A2 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. Defects in COL6A2 are the cause of myosclerosis autosomal recessive (MYOSAR); also known as myosclerotic myopathy or congenital myosclerosis of Lowenthal. A condition characterized by chronic inflammation of skeletal muscle with hyperplasia of the interstitial connective tissue. The clinical picture includes slender muscles with firm 'woody' consistency and restriction of movement of many joints because of muscle contractures. Belongs to the type VI collagen family. 3 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; protein complex; collagen; endoplasmic reticulum lumen; extracellular region; sarcolemma; vesicle

Molecular Function: protein binding

Biological Process: extracellular matrix disassembly; collagen catabolic process; axon guidance; extracellular matrix organization and biogenesis; response to glucose stimulus; cell adhesion

Disease: Bethlem Myopathy; Myosclerosis, Autosomal Recessive; Ullrich Congenital Muscular Dystrophy

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.