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ACVR2B/Activin RIIB  Protein, Antibody, ELISA Kit, cDNA Clone

製品の説明: Active  
発現宿主: Human Cells  
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10229-H02H-100
10229-H02H-200
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発現宿主: Human Cells  
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10229-H08H-100
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200 µg 
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発現宿主: Human Cells  
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50173-M03H-50
50173-M03H-100
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製品の説明: Active  
発現宿主: Human Cells  
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50173-M08H-50
50173-M08H-100
50 µg 
100 µg 
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発現宿主: Human Cells  
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90056-C08H-100
90056-C08H-200
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発現宿主: Human Cells  
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90056-C02H-100
90056-C02H-200
100 µg 
200 µg 
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発現宿主: Human Cells  
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90056-CCCH-50
90056-CCCH-100
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ACVR2B/Activin RIIB 関連経路

ACVR2B/Activin RIIB サマリー & タンパク質情報

ACVR2B/Activin RIIB 背景

遺伝子の概要: Activins are dimeric growth and differentiation factors which belong to the transforming growth factor-beta (TGF-beta) superfamily of structurally related signaling proteins. Activins signal through a heteromeric complex of receptor serine kinases which include at least two type I (I and IB) and two type II (II and IIB) receptors. These receptors are all transmembrane proteins, composed of a ligand-binding extracellular domain with cysteine-rich region, a transmembrane domain, and a cytoplasmic domain with predicted serine/threonine specificity. Type I receptors are essential for signaling; and type II receptors are required for binding ligands and for expression of type I receptors. Type I and II receptors form a stable complex after ligand binding, resulting in phosphorylation of type I receptors by type II receptors. Type II receptors are considered to be constitutively active kinases. ACVR2B gene encodes activin A type IIB receptor, which displays a 3- to 4-fold higher affinity for the ligand than activin A type II receptor. [provided by RefSeq, Jul 2008]
General information above from NCBI
触媒活性: ATP + [receptor-protein] = ADP + [receptor- protein] phosphate.
補因子: Magnesium or manganese (By similarity).
サブユニット構造: Forms an activin receptor complex with activin type II receptors such as ACVR1B. Interacts with VPS39.
細胞内位置: Cell membrane; Single-pass type I membrane protein (By similarity).
翻訳後: Phosphorylated. Constitutive phosphorylation is in part catalyzed by its own kinase activity.
疾患関連性: Heterotaxy, visceral, 4, autosomal (HTX4) [MIM:613751]: A form of visceral heterotaxy, a complex disorder due to disruption of the normal left-right asymmetry of the thoracoabdominal organs. Visceral heterotaxy or situs ambiguus results in randomization of the placement of visceral organs, including the heart, lungs, liver, spleen, and stomach. The organs are oriented randomly with respect to the left-right axis and with respect to one another. It can been associated with variety of congenital defects including cardiac malformations. HTX4 clinical features 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
シーケンスの類似性: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 protein kinase domain.
General information above from UniProt

ACVR2A and ACVR2B are two activin type II receptors. ACVR2B is integral to the activin and myostatin signaling pathway. Ligands such as activin and myostatin bind to ACVR2A and ACVR2B. Myostatin, a negative regulator of skeletal muscle growth, is regarded as a potential therapeutic target and binds to ACVR2B effectively, and to a lesser extent, to ACVR2A. The structure of human ACVR2B kinase domain in complex with adenine establishes the conserved bilobal architecture consistent with all other catalytic kinase domains. Haplotype structure at the ACVR2B and follistatin loci may contribute to interindividual variation in skeletal muscle mass and strength. Defects in ACVR2B are a cause of left-right axis malformations.

ACVR2B/Activin RIIB 代替名

HTX4,ACTRIIB,ActR-IIB, [homo-sapiens]
ACVR2B,ActR-IIB,MGC116908,ACTRIIB, [human]
ActRIIB,Acvr2b,MGC118477, [mouse]
ActRIIB, [mus-musculus]

ACVR2B/Activin RIIB 関連研究

  1. Kosaki R, et al. (1999) Left-right axis malformations associated with mutations in ACVR2B, the gene for human activin receptor type IIB. Am J Med Genet. 82(1):70-6.
  2. Dupont S, et al. (2001) No evidence for linkage or for diabetes-associated mutations in the activin type 2B receptor gene (ACVR2B) in French patients with mature-onset diabetes of the young or type 2 diabetes. Diabetes 50(5):1219-21.
  3. Albertson RC, et al. (2005) Zebrafish acvr2a and acvr2b exhibit distinct roles in craniofacial development. Developmental dynamics 233(4): 1405-18.
  4. Walsh S, et al. (2007) Activin-type II receptor B (ACVR2B) and follistatin haplotype associations with muscle mass and strength in humans. J Appl Physiol. 102(6):2142-8.
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