Rabbit anti-Human COL4A3 Polyclonal Antibody, Biotin conjugated

Uniprot Id

Q01955

Target Species

Human

Target Name

COL4A3

Target Full Name

Collagen alpha-3(IV) chain

Target Function

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.; Tumstatin, a cleavage fragment corresponding to the collagen alpha 3(IV) NC1 domain, possesses both anti-angiogenic and anti-tumor cell activity; these two anti-tumor properties may be regulated via RGD-independent ITGB3-mediated mechanisms.

Target Involvement

Alport syndrome, autosomal recessive (APSAR); Hematuria, benign familial (BFH); Alport syndrome, autosomal dominant (APSAD)

Target Subcellular Location

Secreted, extracellular space, extracellular matrix, basement membrane.

Target Protein Families

Type IV collagen family

Target Tissue Specificity

Alpha 3 and alpha 4 type IV collagens are colocalized and present in kidney, eye, basement membranes of lens capsule, cochlea, lung, skeletal muscle, aorta, synaptic fibers, fetal kidney and fetal lung. PubMed:8083201 reports similar levels of expression

Target Research Area

Cell Adhesion

Target Synonyms

Alpha 3 type IV collagen; Alpha3 type IV collagen; CO4A3_HUMAN; COL4A 3; Col4a3; Collagen alpha 3(IV) chain; Collagen IV alpha 3 polypeptide; Collagen type IV alpha 3 (Goodpasture antigen); Collagen type IV alpha 3; Collagen type IV alpha 3 chain; Goodpasture antigen; OTTHUMP00000195044; Tumstatin

Target Background

Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. 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 so that each gene pair shares a common promoter.