ACVRL1 Rabbit Polyclonal Antibody
Product Data | |
Application | ELISA, IF, IHC, WB |
---|---|
Recommended Dilution | Western Blot: 1/500-1/1000. Immunofluorescence: 1/50-1/200. Immunohistochemistry on Paraffin Sections: 1/50-1/200. |
Reactivity | Human, Mouse, Rat |
Antibody Host | Rabbit |
Clonality | Polyclonal |
Immunogen | Synthetic peptide corresponding to amino acids 200-250 of Human ACVRL1. |
Specificity | This antibody detects endogenous levels of ALK-1 protein (region surrounding Phe231). |
Buffer | Phosphate buffered saline (PBS), pH 7.2 State: Aff - Purified State: Liquid purified Ig fraction (> 95% pure by SDS-PAGE) Preservative: 0.05% Sodium Azide |
Concentration | 1.0 mg/ml |
Purification | Affinity Chromatography using epitope-specific immunogen |
Conjugation | Unconjugated |
Storage | Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Stability | Shelf life: One year from despatch |
Shipping | Blue Ice |
Predicted Protein Size | ~56 kDa |
Gene Name | activin A receptor like type 1 |
Database Link | |
Background | Mutations in two genes, Endoglin (also designated CD105) and ACVRL1 / ALK1 (activin receptorlike 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. |
Synonyms | ACVRLK1, Activin receptor-like kinase 1, SKR3 |
Reference Data |
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