antibody

Anti-ACVRL1 Antibody

MBS416755

0.1 mL

230 USD

polyclonal

rabbit

nonconjugated

western blot, ELISA, immunohistochemistry, enzyme immunoassay

Antibody

Anti-ACVRL1 Antibody

[ACVRL1]

[activin A receptor type II-like kinase 1 variant 4, partial; Serine/threonine-protein kinase receptor R3; serine/threonine-protein kinase receptor R3; TGF-B superfamily receptor type I; activin A receptor, type II-like kinase 1; activin A receptor type II-like 1; Activin receptor-like kinase 1; ALK-1; TGF-B superfamily receptor type I; TSR-I]

[ACVRL1]

[ACVRL1; ACVRL1; HHT; ALK1; HHT2; ORW2; SKR3; ALK-1; TSR-I; ACVRLK1]

ACVL1_HUMAN

Polyclonal

IgG

Rabbit

Human. Not yet tested in other species

89

Affinity purified

Liquid

100ug/100ul

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

ELISA (EIA), Western Blot (WB) Immunohistochemistry (IHC)

Peptide ELISA titer: 1:1,000-1:10,000. Western blot analysis: 1:200-5,000. Immunohistochemistry: 1:100-300. Optimal dilutions/concentrations should be determined by the end user.

Testing Data

Immunohistochemistry

Storage Buffer: PBS, pH 7.4 with 0.02% Sodium Azide. Antibody Type: Primary Antibody

Immunogen: Rabbit polyclonal ACVRL1 (1) antibody was raised against a peptide from near the C terminal residues of human ACVRL1 protein.

This gene encodes a type I cell-surface receptor for the TGF-beta superfamily of ligands. It shares with other type I receptors a high degree of similarity in serine-threonine kinase subdomains, a glycine- and serine-rich region (called the GS domain) preceding the kinase domain, and a short C-terminal tail. The encoded protein, sometimes termed ALK1, shares similar domain structures with other closely related ALK or activin receptor-like kinase proteins that form a subfamily of receptor serine/threonine kinases. Mutations in this gene are associated with hemorrhagic telangiectasia type 2, also known as Rendu-Osler-Weber syndrome 2. [provided by RefSeq, Jul 2008]

¯Ë†1¯°Brakensiek,K., Frye-Boukhriss,H., Malzer,M., et al. Detection of a significant association between mutations in the ACVRL1 gene and hepatic involvement in German patients with hereditary haemorrhagic telangiectasia.Clin. Genet. 74 (2), 171-177 (2008)

300216367

ADJ79923.1

56,124 Da

ALK1 Pathway (137985); ALK1 Signaling Events Pathway (137968); Id Signaling Pathway (198871); TGF-beta Receptor Signaling Pathway (198774)

This gene encodes a type I cell-surface receptor for the TGF-beta superfamily of ligands. It shares with other type I receptors a high degree of similarity in serine-threonine kinase subdomains, a glycine- and serine-rich region (called the GS domain) preceding the kinase domain, and a short C-terminal tail. The encoded protein, sometimes termed ALK1, shares similar domain structures with other closely related ALK or activin receptor-like kinase proteins that form a subfamily of receptor serine/threonine kinases. Mutations in this gene are associated with hemorrhagic telangiectasia type 2, also known as Rendu-Osler-Weber syndrome 2. [provided by RefSeq, Jul 2008]

ALK1: a kinase of the STKR family. A type I cell-surface receptor for the TGF-beta superfamily of ligands including Activin A. Highly expressed in human placenta and lung. Deficiency causes hemorrhagic telangiectasia type 2 also known as Rendu-Osler-Weber syndrome 2. Protein type: EC 2.7.11.30; Membrane protein, integral; Protein kinase, TKL; Kinase, protein; Protein kinase, Ser/Thr (receptor); TKL group; STKR family; Type1 subfamily. Chromosomal Location of Human Ortholog: 12q13.13. Cellular Component: cell surface; cell soma; integral to plasma membrane; dendrite. Molecular Function: metal ion binding; protein kinase binding; transmembrane receptor protein serine/threonine kinase activity; transforming growth factor beta receptor activity; protein serine/threonine kinase activity; protein binding; activin receptor activity, type I; transforming growth factor beta binding; transforming growth factor beta receptor activity, type I; activin binding; SMAD binding; ATP binding; receptor signaling protein serine/threonine kinase activity. Biological Process: blood circulation; protein heterooligomerization; positive regulation of transcription, DNA-dependent; activin receptor signaling pathway; negative regulation of cell adhesion; regulation of blood vessel endothelial cell migration; signal transduction; protein amino acid phosphorylation; BMP signaling pathway; negative regulation of cell proliferation; positive regulation of endothelial cell differentiation; negative regulation of endothelial cell differentiation; regulation of transcription, DNA-dependent; transforming growth factor beta receptor signaling pathway; regulation of blood pressure; lymphangiogenesis; angiogenesis; negative regulation of cell migration; positive regulation of BMP signaling pathway; regulation of DNA replication; regulation of endothelial cell proliferation; in utero embryonic development; negative regulation of focal adhesion formation; negative regulation of blood vessel endothelial cell migration; blood vessel maturation; positive regulation of angiogenesis; blood vessel endothelial cell proliferation during sprouting angiogenesis; positive regulation of chondrocyte differentiation; negative regulation of endothelial cell proliferation; positive regulation of endothelial cell proliferation; blood vessel remodeling; positive regulation of transcription from RNA polymerase II promoter; negative regulation of cell growth; wound healing, spreading of epidermal cells. Disease: Telangiectasia, Hereditary Hemorrhagic, Type 2

0.1 mL

230 USD