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.
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000ELISA: 1:5000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human Cleaved-COL4A3 (L1425).
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to aa1350-1430 of human COL4A3 at C-terminal.
