Cleaved-Tumstatin (P1426) Polyclonal Antibody

Ab type

Primary antibody

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. COL4A3 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. COL4A3 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, COL4A3 is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter.

Reactivity

Human;Monkey

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Immunogen

The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:1407-1456

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

161813

synonyms

COL4A3; Collagen alpha-3(IV) chain; Goodpasture antigen