Serine/threonine-Protein Kinase Receptor R3 (ACVRL1) Antibody
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Description
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.
Documents del producto
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Serine/threonine-Protein Kinase Receptor R3 (ACVRL1) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | WB: 1/1000, IHC-P: 1/50 - 1/100, FCM: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS. |
| Size 1 | 80 µl |
| Size 2 | 400 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IHC, FCM |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P37023 |
| Alias | Activin receptor-like kinase 1,ALK-1,SKR3 |
| Background | Antibody anti-ACVRL1 |
| Status | RUO |
Descripción
ACVRL1, also known as ALK1, is a type I receptor that primarily binds BMP9 and BMP10. It is critical for vascular development and endothelial cell function. ACVRL1 regulates angiogenesis through the SMAD1/5/8 signaling pathway, promoting endothelial proliferation and vessel stability. Mutations in ACVRL1 cause hereditary hemorrhagic telangiectasia (HHT), a genetic disorder characterized by abnormal blood vessel formation and recurrent bleeding. It is primarily expressed in vascular endothelial cells.
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Type I receptor for TGF-beta family ligands BMP9/GDF2 and BMP10 and important regulator of normal blood vessel development. 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. May bind activin as well.
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Serine/threonine-Protein Kinase Receptor R3 (ACVRL1) Antibody
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.
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Serine/threonine-Protein Kinase Receptor R3 (ACVRL1) Antibody
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.
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