Anti-COL4A3 Antibody

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.

Expressed in adipose tissue, heart, brain, skeletal muscle, pancreas, thymus, prostate, testis, ovary, small intestine and colon. Highest levels in adipose tissue, small intestine and colon.

Amino Acid Metabolism, Amino Acids, Cancer, Energy Metabolism, Energy Transfer Pathways, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Signal Transduction

Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) . Can act as a tumor suppressor.

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.

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