ALK-1 抗体 (RM0015-1B03): sc-101556

ALK-1 Antibody (RM0015-1B03) is an IgG₂ rat monoclonal ALK-1 antibody (also designated ACVRL1 antibody, ALK1 antibody, SKR3 antibody, or TSR-I antibody) that detects the ALK-1 protein of mouse, rat and human origin by WB, IP and IF. ALK-1 Antibody (RM0015-1B03) is available as the non-conjugated anti-ALK-1 antibody. Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by vascular abnormalities such as dilated vessels, hemorrhages, liver and lung congestion, and brain or heart ischemia. Mutations in two genes, Endoglin (also designated CD105) and ALK-1 (activin receptor-like kinase 1, also designated TGFβ Superfamily RI), are responsible for HHT. Endoglin is mutated in HHT1, and ALK-1 is mutated in HHT2, both of which are thought to be caused by haploinsufficiency. Endoglin and ALK-1 are type III and type I members of the TGFβ receptor superfamily, respectively, that are expressed on vascular endothelial cells. Endoglin can only bind ligands of the TGFβ superfamily via association with the respective ligand binding receptors for TGFβ1, TGFβ3, Activin-A, BMP-2 and BMP-7. ALK-1 preferentially binds TGFβ1 and is expressed in bone marrow stromal cells, lung, brain, kidney and spleen.

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References:

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5. Expression and structural features of endoglin (CD105), a transforming growth factor beta1 and beta3 binding protein, in human melanoma.  |  Altomonte, M., et al. 1996. Br J Cancer. 74: 1586-91. PMID: 8932339
6. Mutation and expression analysis of the endoglin gene in hereditary hemorrhagic telangiectasia reveals null alleles.  |  Gallione, CJ., et al. 1998. Hum Mutat. 11: 286-94. PMID: 9554745