ACVRL1, Polyclonal Antibody

Serine/threonine-protein kinase receptor R3; SKR3; Activin receptor-like kinase 1; ALK-1; TGF-B superfamily receptor type I; TSR-I; ACVRL1; ACVRLK1; ALK1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This ACVRL1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 38-68 amino acids from the N-terminal region of human ACVRL1.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ACVRL1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ACVRL1 is 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. This 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.

Cancer; Cardiovascular; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS), Immunohistochemistry (IHC)

WB~~1:1000

Western blot of ACVRL1 Pab. TOP LEFT: Mouse heart tissue lysate.

Western blot analysis of ACVRL1 (arrow) using rabbit polyclonal ACVRL1 Antibody (N-term). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the ACVRL1 gene (Lane 2) (Origene Technologies).

Flow cytometric analysis of HepG2 cells using ACVRL1 Antibody (N-term) (bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma.

human chondrocytes (C28/I2 cells), transfected with empty vector (lane 1, 3) or ACVRL1(lane 2, 4). RIPA lysis buffer, 20 ug/lane of protein, primary antibody dilution 1:1000, blocking solution is 5% milk in TBST (lane 1 and 2), 5% BSA in TBST (lane 3 and 4). Data courtesy of Kenneth Finnson, Montreal General Hospital.

56124

activin A receptor type II-like 1

serine/threonine-protein kinase receptor R3

Serine/threonine-protein kinase receptor R3

ACVRLK1; ALK1; SKR3; ALK-1; TSR-I  [Similar Products]

ACVL1_HUMAN

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: Protein kinase, TKL; Kinase, protein; EC 2.7.11.30; Membrane protein, integral; 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: protein heterooligomerization; blood circulation; 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; blood vessel endothelial cell proliferation during sprouting angiogenesis; positive regulation of angiogenesis; positive regulation of chondrocyte differentiation; negative regulation of endothelial cell proliferation; blood vessel remodeling; positive regulation of endothelial cell proliferation; 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

Strausberg, R.L., et al., Proc. Natl. Acad. Sci. U.S.A. 99(26):16899-16903 (2002).
Berg, J.N., et al., Am. J. Hum. Genet. 61(1):60-67 (1997).
Johnson, D.W., et al., Nat. Genet. 13(2):189-195 (1996).
ten Dijke, P., et al., Oncogene 8(10):2879-2887 (1993).
Attisano, L., et al., Cell 75(4):671-680 (1993).

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