Anti-ACTR1抗体Members of the transforming growth factor b superfamily bind to a pair of transmembrane proteins, known as receptor types I and II,

产品编号 yb-12415R
英文名称 Anti-ACTR1抗体
中文名称 激活素受体1A抗体
别    名 Activin Receptor Type IA; Activin A receptor type I; Activin A receptor type II like kinase 2; Activin receptor type I; Activin receptor type-1; Activin receptor-like kinase 2; ACTR1; ACTR 1; ACTR-1; ACTR-I; ACTRI; ACVR 1; ACVR1; ACVR1_HUMAN; ACVR1A; ACVRLK 2; ACVRLK2; ALK 2; ALK-2; ALK2; FOP; Hydroxyalkyl protein kinase; Serine/threonine protein kinase receptor R1; Serine/threonine-protein kinase receptor R1; SKR 1; SKR1; TGF-B superfamily receptor type I; TGFB superfamily receptor type I; TSR-I; TSRI; TSRI.
Anti-ACTR1抗体    
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  信号转导  生长因子和激素  转录调节因子  激酶和磷酸酶  细胞膜受体  跨膜蛋白  细胞膜蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human SKR1/ACTR1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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.

PubMed PubMed
产品介绍 background:
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).

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 activin. May be involved for left-right pattern formation during embryogenesis.

Subunit:
Interacts with FKBP1A. Interacts with FCHO1.

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

Tissue Specificity:
Expressed in normal parenchymal cells, endothelial cells, fibroblasts and tumor-derived epithelial cells.

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.

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.

Database links:
UniProtKB/Swiss-Prot: Q04771.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.