Rabbit anti-Human COL4A6 Polyclonal Antibody | anti-COL4A6 antibody

COL4A6 (G1223) Polyclonal Antibody

Cat. Num. AAA3013515

• Gene Names: COL4A6; DFNX6; DELXq22.3; CXDELq22.3
• Reactivity: Human
• Applications: 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: COL4A6; Polyclonal Antibody; COL4A6 (G1223) Polyclonal Antibody; Collagen Alpha 6(IV); Collagen alpha 6(IV) chain; Collagen IV alpha 6 polypeptide; Collagen of basement membrane alpha 6; Collagen type IV alpha 6; dJ889N15.4; MGC88184; Type IV alpha 6 collagen; anti-COL4A6 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: COL4A6 (G1223) polyclonal antibody detects endogenous levels of COL4A6 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: 1691

• Applicable Applications for anti-COL4A6 antibody: Immunohistochemistry (IHC)
• Application Notes: IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 1190-1240 of Human COL4A6.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Related Product Information for anti-COL4A6 antibody

Collagens are fibrous, extracellular matrix proteins with high tensile strength and are the major components of connective tissue, such as tendons and cartilage. Collagens contain triple helix domains and frequently show lateral selfassociation in order to form complex connective tissues. Several collagens play a role in cell adhesion, which is important for maintaining normal tissue architecture and function. COL4A6 (collagen, type IV, alpha 6) is a 1, 691 amino acid secreted protein belonging to the type IV collagen family. Type IV collagen, a major component of the basement membrane (BM), is composed of six genetically distinct alpha(IV) chains, alpha1(IV) to alpha6(IV). Defects in the gene encoding COL4A6 is believed is the cause of X-linked Alport syndrome (AS), which is characterized by macroscopic hematuria, cataracts and leiomyomatosis.

NCBI and Uniprot Product Information

• NCBI GI #: 148536823
• NCBI GeneID: 1288
• NCBI Accession #: NP_001838.2
• NCBI GenBank Nucleotide #: NP_001838.2
• UniProt Accession #: Q14031
• Molecular Weight: ~ 170kDa
• NCBI Official Full Name: collagen alpha-6(IV) chain isoform A
• NCBI Official Synonym Full Names: collagen type IV alpha 6 chain
• NCBI Official Symbol: COL4A6
• NCBI Official Synonym Symbols: DFNX6; DELXq22.3; CXDELq22.3
• NCBI Protein Information: collagen alpha-6(IV) chain
• UniProt Protein Name: Collagen alpha-6(IV) chain
• Protein Family: Collagen
• UniProt Gene Name: COL4A6
• UniProt Entry Name: CO4A6_HUMAN

NCBI Description

This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene, alpha 5 type IV collagen, so that the gene pair shares a common promoter. Deletions in the alpha 5 gene that extend into the alpha 6 gene result in diffuse leiomyomatosis accompanying the X-linked Alport syndrome caused by the deletion in the alpha 5 gene. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2013]

Uniprot Description

COL4A6: Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS). Belongs to the type IV collagen family. 2 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: Xq22

Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region

Molecular Function: extracellular matrix structural constituent

Biological Process: collagen catabolic process; extracellular matrix disassembly; extracellular matrix organization and biogenesis; cell adhesion

Disease: Deafness, X-linked 6