Anti-COL4A3 Antibody

SKU: A01755-3

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: IHC,ELISA

Application Details: Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: COL4A3

Specificity:

Background: Collagen alpha-3(IV) chain is a protein that in humans is encoded by the COL4A3 gene. 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.

Immunogen: E.coli-derived human COL4A3 recombinant protein (Position: P1426-H1670).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Badenas, C., Praga, M., Tazon, B., Heidet, L., Arrondel, C., Armengol, A., Andres, A., Morales. E., Camacho, J. A., Lens, X., Davila, S., Mila, M., Antignac, C., Darnell, A., Torra, R. Mutations in the COL4A4 and COL4A3 genes cause familial benign hematuria. J. Am. Soc. Nephrol. 13: 1248-1254, 2002. 2. Bernal, D., Quinones, S., Saus, J. The human mRNA encoding the Goodpasture antigen is alternatively spliced. J. Biol. Chem. 268: 12090-12094, 1993. 3. Butkowski, R. J., Langeveld, J. P. M., Wieslander, J., Hamilton, J., Hudson, B. G. Localization of the Goodpasture epitope to a novel chain of basement membrane collagen. J. Biol. Chem. 262: 7874-7877, 1987.

Uniprot ID: Q01955

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 130 kDa

Calculated Molecular Weight:

Gene ID: 1285

Protein Name: Collagen alpha-3

Gene Full Name: collagen type IV alpha 3 chain

Synonyms: COL4A3; Tumstatin; Goodpasture antigen; Collagen alpha-3(IV) chain; ATS2; ATS3; ATS3A; ATS3B; BFH2