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Rabbit Anti-Activin receptor type-1/APC Conjugated antibody (bs-12415R-APC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12415R-APC
英文名稱 Rabbit Anti-Activin receptor type-1/APC Conjugated antibody
中文名稱 APC標記的激活素受體1A抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉導  生長因子和激素  轉錄調節因子  激酶和磷酸酶  細胞膜受體  跨膜蛋白  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SKR1/ACTR1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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:


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