Rabbit anti-Human COL6A2 Polyclonal Antibody | anti-COL6A2 antibody

COL6A2 (Y711) Polyclonal Antibody

Cat. Num. AAA3002246

• Gene Names: COL6A2; UCMD1; BTHLM1; PP3610
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: COL6A2; Polyclonal Antibody; COL6A2 (Y711) Polyclonal Antibody; Collagen alpha 2(VI) chain; Collagen alpha-2(VI) chain; Collagen VI alpha 2 polypeptide; PP3610; anti-COL6A2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: COL6A2 (Y711) polyclonal antibody detects endogenous levels of COL6A2 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 1019

• Applicable Applications for anti-COL6A2 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 681-730 of Human COL6A2.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of COL6A2 (Y711) pAb at 1:1000 dilutionLane1:U-87MG whole cell lysate (40ug) Lane2:PC3 whole cell lysate (40ug) Lane3:MCF-7 whole cell lysate (40ug))

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of COL6A2 (Y711) pAb in paraffin-embedded human breast carcinoma tissue at 1:100.)

Related Product Information for anti-COL6A2 antibody

Collagens (COLs) are fibrous, extracellular matrix proteins with high tensile strength that function as the major components of connective tissue, such as tendons and cartilage. All COL proteins contain a triple helix domain and frequently show lateral self-association in order to form complex connective tissues. Existing as a trimer with two other type VI alpha proteins, COL6A2 acts as a cell-binding protein that plays an important role in the organization of matrix components. Defects in the gene encoding COL6A2 are associated with Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). Multiple isoforms of COL6A2 exist due to alternative splicing events.

NCBI and Uniprot Product Information

• NCBI GI #: 115527062
• NCBI GeneID: 1292
• NCBI Accession #: NP_001840.3
• NCBI GenBank Nucleotide #: NP_001840.3
• UniProt Accession #: P12110
• Molecular Weight: ~109kDa
• NCBI Official Full Name: collagen alpha-2(VI) chain isoform 2C2
• NCBI Official Synonym Full Names: collagen type VI alpha 2 chain
• NCBI Official Symbol: COL6A2
• NCBI Official Synonym Symbols: UCMD1; BTHLM1; PP3610
• NCBI Protein Information: collagen alpha-2(VI) chain
• UniProt Protein Name: Collagen alpha-2(VI) chain
• Protein Family: Collagen
• UniProt Gene Name: COL6A2
• UniProt Entry Name: CO6A2_HUMAN

NCBI Description

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]

Uniprot Description

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: Secreted; Secreted, signal peptide; Extracellular matrix

Chromosomal Location of Human Ortholog: 21q22.3

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

Molecular Function: protein binding

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

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