A25916
Collagen IV Rabbit monoclonal antibody
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- SKU:
- A25916
- Zusätzliche Namen:
- ATS2|ATS3|ATS3A|ATS3B|BFH2|BSVD|BSVD1|COL4A1s|PADMAL|RATOR
- Anwendung:
- ELISA, WB, IF, ICC
- Molekulargewicht:
- 162 kDa
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Abclonal
- Host:
- Rabbit
- Reaktivitäten:
- Human
- Immunogen:
- Recombinant protein (or fragment).This information is considered to be commercially sensitive.
- Formulierung:
- Unmodified
- Sequenz:
- AIAIAVHSQTTDIPPCPHGWISLWKGFSFIMFTSAGSEGTGQALASPGSCLEEFRASPFLECHGRGTCNYYSNSYSFWLASLNPERMFRKPIPSTVKAGELEKIISRCQVCMKKR
- Uniprot:
- P02462, P08572, P29400, Q01955
- Synonyme:
- arresten;ASLN;ATS;ATS1;ATS2;ATS3;BFH;BSVD;BSVD1;BSVD2;CA44;CA54;canstatin;COL4A1 NC1 domain;COL4A1s;collagen alpha-1(IV) chain;collagen alpha-2(IV) chain;collagen alpha-3(IV) chain;collagen alpha-4(IV) chain;collagen alpha-5(IV) chain;collagen IV, alpha-1 polypeptide;collagen IV, alpha-3 polypeptide;Collagen IV, alpha-4 polypeptide;collagen IV, alpha-5 polypeptide;collagen of basement membrane, alpha-1 chain;collagen of basement membrane, alpha-4 chain;collagen of basement membrane, alpha-5 chain;collagen, type IV, alpha 3 (Goodpasture antigen);collagen, type IV, alpha 4;collagen, type IV, alpha 5;dA149D17.3;dA24A23.1;Goodpasture antigen;ICH;PADMAL;POREN2;RATOR;tumstatin
- Weitere Details:
- 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. This gene encodes a type IV collagen alpha protein. Type IV collagen proteins are integral components of basement membranes. This gene shares a bidirectional promoter with a paralogous gene on the opposite strand. The protein consists of an amino-terminal 7S domain, a triple-helix forming collagenous domain, and a carboxy-terminal non-collagenous domain. It functions as part of a heterotrimer and interacts with other extracellular matrix components such as perlecans, proteoglycans, and laminins. In addition, proteolytic cleavage of the non-collagenous carboxy-terminal domain results in a biologically active fragment known as arresten, which has anti-angiogenic and tumor suppressor properties. Mutations in this gene cause porencephaly, cerebrovascular disease, and renal and muscular defects. Alternative splicing results in multiple transcript variants.
- Versandbedingungen:
- Blue Ice