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 r
Catalog No
L0227
Reactivity
Human, Rat, Mouse
Applications
WB, ELISA
Modification
Cleaved Specific
Source
Polyclonal Rabbit
Dilution
Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:1376-1425
Specificity
Cleaved-COL4A3 (L1425) Polyclonal Antibody detects endogenous levels of fragment of activated COL4A3 protein resulting from cleavage adjacent to L1425.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Western blot analysis of lysates from 293 cells, treated with etoposide 25uM 1h, using Collagen IV alpha3 (Cleaved-Leu1425) Antibody. The lane on the right is blocked with the synthesized peptide.