Rabbit anti-Human ALK-1 Monoclonal Antibody | anti-ALK-1 antibody

ALK-1

Cat. Num. AAA370004

• Gene Names: ACVRL1; HHT; ALK1; HHT2; ORW2; SKR3; ALK-1; TSR-I; ACVRLK1
• Reactivity: Human
• Synonyms: ALK-1; Monoclonal Antibody; anti-ALK-1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG
• Clone Number: RBT-ALK1
• Form/Format: Anti-ALK-1 is a rabbit monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH 7.5, containing BSA and sodium azide as a preservative.

• Control: Anaplastic Large Cell Lymphoma
• Localization: Cytoplasmic, Nuclear
• Reactivity Note: Paraffin, Frozen
• Preparation and Storage: Store at 2 to 8 degree C in the dark.

Dilution Information

Immunohistochemistry (IHC)

Related Product Information for anti-ALK-1 antibody

Anaplastic Lymphoma Kinase (ALK) was originally discovered as a NPM (Nucleophosmin)-ALK fusion protein. The ALK gene is on chromosome 2. Upon translocation between chromosome 2 and chromosome 5 t(2;5), the ALK gene fuses with the NPM gene. The chimeric product (NPM ALK) resulting from t(2;5) translocation is a protein of 80 kDa with the N terminal portion of NPM linked to the complete intracellular portion of ALK.This antibody recognizes a human p80 protein, identified as a hybrid of the Anaplastic Lymphoma Kinase (ALK) gene and the Nucleophosmin (NPM) gene resulting from the t(2;5)(p23;q35) translocation found in a third of Large-Cell Lymphomas. ALK-1 is detected in 60% of Anaplastic Large-Cell Lymphomas and has proven to indicate a better prognosis in the ALK-1 (+) group.

NCBI and Uniprot Product Information

• NCBI GI #: 402197
• NCBI GeneID: 94
• UniProt Accession #: P37023; A6NGA8
• Molecular Weight: 56,124 Da
• NCBI Official Full Name: ALK-1
• NCBI Official Synonym Full Names: activin A receptor type II-like 1
• NCBI Official Symbol: ACVRL1
• NCBI Official Synonym Symbols: HHT; ALK1; HHT2; ORW2; SKR3; ALK-1; TSR-I; ACVRLK1
• NCBI Protein Information: serine/threonine-protein kinase receptor R3; activin receptor-like kinase 1; TGF-B superfamily receptor type I; activin A receptor, type II-like kinase 1
• UniProt Protein Name: Serine/threonine-protein kinase receptor R3
• UniProt Gene Name: ACVRL1
• UniProt Synonym Gene Names: ACVRLK1; ALK1
• UniProt Entry Name: ACVL1_HUMAN

NCBI Description

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]

Uniprot Description

Function: On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. May bind activin as well.

Catalytic activity: ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.

Cofactor: Magnesium or manganese

Subcellular location: Membrane; Single-pass type I membrane protein.

Involvement in disease: Defects in ACVRL1 are the cause of hereditary hemorrhagic telangiectasia type 2 (HHT2) [

MIM:600376]; also known as Osler-Rendu-Weber syndrome 2 (ORW2). HHT2 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary, cerebral and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia. Ref.3 Ref.7 Ref.8 Ref.9 Ref.10 Ref.11 Ref.12 Ref.14 Ref.15

Sequence similarities: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.Contains 1 GS domain.Contains 1 protein kinase domain.