Product information
Price | €175, €275, €395 |
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Size | 20 µl/µg | 50 µl/µg | 100 µl/µg |
Short name | COL4A3 Antibody |
Host | Rabbit |
Isotype | IgG |
Target protein | COL4A3 |
Recommended applications | ELISA, IF, IHC-p |
Reactivity | Human |
Immunogen | The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:801-850 |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
Formulation | Liquid in PBS containing 50% glycerol; 0.5% BSA and 0.02% sodium azide. |
Storage | -20°C for 1 year |
Product code | MB-AP11132 |
Additional information
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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.
COL4A3, Collagen alpha-3(IV) chain, Goodpasture antigen
Human: Q01955, Mouse: Q9QZS0
Human: 1285