Product Overview
Description
CLPP-00150247 is recombinant human ACVR2B protein
Applications
HPLC, SDS-PAGE
Protein Length
Protein fragment
Nature
Recombinant Protein
Endotoxin Level
< 1.000 Eu/µg
Sequence
SGRGEAETRECIYYNANWELERTNQSGLERCEGEQDKRLHCYASWRNSSGTIELVKKGCWLDDFNCYDRQECVATEENPQVYFCCCEGNFCNERFTHLPEAGGPEVTYEPPPTAPTVDHHHHHH
Sequence Similarities
Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. Contains 1 protein kinase domain.
Predicted Molecular Weight
14 kDa including tags
Target Information
Alternative Names
Activin A receptor type IIB; Activin receptor type 2B; Activin receptor type IIB; Activin receptor type-2B; ActR IIB; ACTR-IIB; ActRIIB; ACVR 2B; ACVR2B; AVR2B_HUMAN; HTX4; MGC116908
Protein 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.
Involvement in Disease
Defects in ACVR2B are the cause of visceral heterotaxy autosomal type 4 (HTX4). 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.
Shipping & Handling
Constituents
99% Phosphate Buffer, 0.88% Sodium chloride.
Shipping
Shipped at 4 °C.
Storage
Store at -20 °C for long term.
