COL4A3 Antibody

Catalog Number:

37058

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#: Q01955
NCBI Gene ID: 1285
Gene Accssion: NP_000082

Form of Antibody:

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

Storage/Stability:

Immunogen:

Synthetic peptide corresponding to a region derived from internal residues of human collagen, type IV, alpha 3 (Goodpasture antigen)

Purification:

Antigen affinity purification.

Specificity/Sensitivity:

The antibody detects endogenous levels of total COL4A3 protein.

Applications:

IHC

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.

References:

appl_detail:

Immunohistochemistry: 1:25-1:100

Datasheet PDF