Description
Product Usage Information
Western Blotting: 1:1000
Simple Western™: 1:10 - 1:50
Immunoprecipitation: 1:200
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunoprecipitation
Specificity / Sensitivity
Activin Receptor Type 1 (F6M1E) Rabbit mAb recognizes endogenous levels of total activin receptor type 1 protein. This antibody does not cross-react with activin receptor-like type 1 (ACVRL1/ALK1), or TGF-β Receptor I, II, or III.
Species Reactivity: Human, Mouse, Rat, Monkey
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val370 of human activin receptor type 1.
Background
Activin receptor type 1 (ACVR1), also known as activin receptor-like kinase 2 (ALK2), is a membrane-associated serine/threonine kinase receptor that binds activin A and related TGF-β superfamily growth factors, including bone morphogenetic protein 2 (BMP2), BMP4, BMP6, and BMP7 (1-3). Functional BMP receptor tetramers are composed of a pair of type I (ACVR1, ACVRL1, BMPR1A, or BMPR1B) and a pair of type II (BMPR2, ACVR2A, ACVR2B) receptor proteins (4). Following ligand binding, ACVR1 is phosphorylated by its type II binding partner, which allows recruitment and activation of receptor-regulated SMAD (R-Smad) proteins (5). Phosphorylation of R-Smad proteins results in their translocation to the nucleus, where R-Smads stimulate transcription of target genes (6). Mutations in the corresponding gene, including Arg206His (R206H) and Glu207Asp (Q207D), cause constitutive activation of the receptor and are associated with the genetic developmental disorders hereditary hemorrhagic telangiectasia and fibrodysplasia ossificans progressive (FOP) (7-11). In a mouse model of FOP, overexpression of the wild-type gene lessened the skeletal lesions associated with the R206H genetic background (12). Small molecule inhibitors of the ACVR1 kinase domain can disrupt downstream SMAD1/5 activation, offering a promising therapeutic avenue in FOP, cancers, or other disorders arising from gain-of-function mutations (13,14).
Alternate Names
activin A receptor type 1; activin A receptor, type I; activin A receptor, type II-like kinase 2; Activin receptor type I; Activin receptor type-1; Activin receptor-like kinase 2; ACTR-I; ACTRI; ACVR1; ACVR1A; ACVRLK2; ALK-2; ALK2; FOP; hydroxyalkyl-protein kinase; Serine/threonine-protein kinase receptor R1; SKR1; TGF-B superfamily receptor type I; TSR-I; TSRI
