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Rabbit Anti-Activin Receptor Type IIB/FITC Conjugated antibody
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 A, activin B and inhibin A.
Subunit:
Forms an activin receptor complex with activin type II receptors such as ACVR1B. Interacts with VPS39.
Subcellular Location:
Cell membrane; Single-pass type I membrane protein.
Post-translational modifications:
Phosphorylated. Constitutive phosphorylation is in part catalyzed by its own kinase activity.
DISEASE:
Defects in ACVR2B are the cause of visceral heterotaxy autosomal type 4 (HTX4) [MIM:613751]. A form of visceral heterotaxy, a complex disorder due to disruption of the normal left-right asymmetry of the thoracoabdominal organs. It results in an abnormal arrangement of visceral organs, and a wide variety of congenital defects. Clinical features of visceral heterotaxy type 4 include dextrocardia, right aortic arch and a right-sided spleen, anomalies of the inferior and the superior vena cava, atrial ventricular canal defect with dextro-transposed great arteries, pulmonary stenosis, polysplenia and midline liver.
Similarity:
Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family.
TGFB receptor subfamily.
Contains 1 protein kinase domain.
Database links:
Entrez Gene: 93 Human
Entrez Gene: 11481 Mouse
Entrez Gene: 25366 Rat
Omim: 602730 Human
SwissProt: Q13705 Human
SwissProt: P27040 Mouse
SwissProt: P38445 Rat
Unigene: 174273 Human
Unigene: 390239 Mouse
Unigene: 2448 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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