ACVRL1 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P37023 |
| Other Accession | P37023 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 56124 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human ACVRL1 |
| Epitope Specificity | 21-120/503 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. Contains 1 GS domain.Contains 1 protein kinase domain. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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. |
| Gene ID | 94 |
|---|---|
| Other Names | ACVRLK1; ALK-1; ALK1; HHT; HHT2; ORW2; SKR3; TSR-I; ACVL1_HUMAN; ACVRL1; Activin receptor-like kinase 1 (ALK-1); Serine/threonine-protein kinase receptor R3 (SKR3); TGF-B superfamily receptor type I (TSR-I); 2.7.11.30; activin A receptor like type 1; activin A receptor type II-like 1; activin A receptor type IL; activin receptor-like kinase 1 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | ACVRL1 |
|---|---|
| Synonyms | ACVRLK1, ALK1 |
| Function | 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. |
| Cellular Location | Cell membrane; Single-pass type I membrane protein |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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