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Home > Product > Antibody > Rabbit Anti-Activin receptor type-1/FITC Conjugated antibody
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; ACVR
Cat:
SL12415R-FITC
Species Reactivity：
(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Sheep,)
Immunogen：
KLH conjugated synthetic peptide derived from human SKR1/ACTR1
Format：
Lyophilized or Liquid
Storage instructions：
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 ant
Buffer：
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration：
1mg/ml
Clonality：
Polyclonal
Isotype：
IgG
Applications：
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host：
Rabbit
Calculated MW：
55kDa
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Product PDFs
Datasheet:


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:

Entrez Gene: 90 Human

Entrez Gene: 11477 Mouse

Entrez Gene: 79558 Rat

Omim: 102576 Human

SwissProt: Q04771 Human

SwissProt: P37172 Mouse

SwissProt: P80201 Rat

Unigene: 470316 Human

Unigene: 689 Mouse

Unigene: 87899 Rat




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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