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Cleaved-COL4A3 (L1425) rabbit pAb

Ref. EK-ES1036

50µl
188.00 €
100µl
316.00 €
Estimated delivery in United States, on Wednesday 8 Jan 2025

Product Information

Name:
Cleaved-COL4A3 (L1425) rabbit pAb
Synonyms:
  • COL4A3; Collagen alpha-3(IV) chain; Goodpasture antigen
Description:

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

Notice:
Our products are intended for lab use only. For any other use, please contact us.
Brand:
ELK Biotechnology
Long term storage:
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Hazard Info

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