Activin receptor type-1 Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q04771 |
| Other Accession | Q04771 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 57153 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human Activin receptor type-1 |
| Epitope Specificity | 65-160/509 |
| 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. |
| SUBUNIT | Interacts with FKBP1A. Interacts with FCHO1. |
| DISEASE | Defects in ACVR1 are a cause of fibrodysplasia ossificans progressiva (FOP) [MIM:135100]. FOP is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Heterotopic ossification in FOP begins in childhood and can be induced by trauma or may occur without warning. Bone formation is episodic and progressive, leading to extra-articular ankylosis of all major joints of the axial and appendicular skeleton, rendering movement impossible. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Members of the transforming growth factor b superfamily bind to a pair of transmembrane proteins, known as receptor types I and II, which contain serine/threonine kinases and associate to form a signaling complex (1). Activin has been shown to bind a heteromeric noncovalent complex, which consists of a type I receptor, ACTR-IA (also designated ACVRI and ALK-2) or ACTR-IB (also designated ALK-4 and SKR2), and a type II receptor, ACTR-IIA (also designated ACVR2A) or ACTR-IIB (also designated ACVR2B) (1–6). Both receptor types are highly expressed in brain (5). The activin receptor family members are thought to mediate distinct effects on gene expression, cell differentiation, and morpho- genesis in a dose dependent fashion (5,6). |
| Gene ID | 90 |
|---|---|
| Other Names | ACTRI; ACVR1A; ACVRLK2; ALK2; FOP; SKR1; TSRI; ActR-I; ActRIA; Acvr; Alk-2; Alk8; D330013D15Rik; Tsk7L; ACVR1_HUMAN; ACVR1; Activin receptor type I (ACTR-I); Activin receptor-like kinase 2 (ALK-2); Serine/threonine-protein kinase receptor R1 (SKR1); TGF-B superfamily receptor type I (TSR-I); 2.7.11.30; ACVR1_MOUSE; TSK-7L; Tgfb1; ACVR1_RAT; activin A receptor type 1; activin A receptor, type I |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| 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 | ACVR1 |
|---|---|
| Synonyms | ACVRLK2 |
| Function | Bone morphogenetic protein (BMP) type I receptor that is involved in a wide variety of biological processes, including bone, heart, cartilage, nervous, and reproductive system development and regulation (PubMed:20628059, PubMed:22977237). As a type I receptor, forms heterotetrameric receptor complexes with the type II receptors AMHR2, ACVR2A or ACVR2B (PubMed:17911401). Upon binding of ligands such as BMP7 or GDF2/BMP9 to the heteromeric complexes, type II receptors transphosphorylate ACVR1 intracellular domain (PubMed:25354296). In turn, ACVR1 kinase domain is activated and subsequently phosphorylates SMAD1/5/8 proteins that transduce the signal (PubMed:9748228). In addition to its role in mediating BMP pathway-specific signaling, suppresses TGFbeta/activin pathway signaling by interfering with the binding of activin to its type II receptor (PubMed:17911401). Besides canonical SMAD signaling, can activate non-canonical pathways such as p38 mitogen-activated protein kinases/MAPKs (By similarity). May promote the expression of HAMP, potentially via its interaction with BMP6 (By similarity). |
| Cellular Location | Membrane; Single-pass type I membrane protein. |
| Tissue Location | Expressed in normal parenchymal cells, endothelial cells, fibroblasts and tumor-derived epithelial cells |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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