Anti-eIF2B3抗体The protein encoded by this gene is one of the subunits of initiation factor eIF2B, which catalyzes the exchange

产品编号 yb-14537R
英文名称 Anti-eIF2B3抗体
中文名称 eIF2Bγ蛋白抗体
别    名 EI2BG_HUMAN; EIF 2B; eIF 2B GDP GTP exchange factor subunit gamma; eIF-2B GDP-GTP exchange factor subunit gamma; eIF2B-gamma; Eif2b3; EIF2Bgamma; Eukaryotic translation initiation factor 2B subunit 3 gamma; Eukaryotic translation initiation factor 2B, gamma; eukaryotic translation initiation factor 2B, subunit 3 gamma, 58kDa; OTTHUMP00000010262; OTTHUMP00000010263; Translation initiation factor eIF-2B subunit gamma.
Anti-eIF2B3抗体
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  神经生物学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 50kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human eIF2B3
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
The protein encoded by this gene is one of the subunits of initiation factor eIF2B, which catalyzes the exchange of eukaryotic initiation factor 2-bound GDP for GTP. It has also been found to function as a cofactor of hepatitis C virus internal ribosome entry site-mediated translation. Mutations in this gene have been associated with leukodystrophy with vanishing white matter. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]

Function:
Catalyzes the exchange of eukaryotic initiation factor 2-bound GDP for GTP.

Subunit:
Complex of five different subunits; alpha, beta, gamma, delta and epsilon.

DISEASE:
Defects in EIF2B3 are a cause of leukodystrophy with vanishing white matter (VWM) [MIM:603896]. VWM is a leukodystrophy that occurs mainly in children. Neurological signs include progressive cerebellar ataxia, spasticity, inconstant optic atrophy and relatively preserved mental abilities. The disease is chronic-progressive with, in most individuals, additional episodes of rapid deterioration following febrile infections or minor head trauma. While childhood onset is the most common form of the disorder, some severe forms are apparent at birth. A severe, early-onset form seen among the Cree and Chippewayan populations of Quebec and Manitoba is called Cree leukoencephalopathy. Milder forms may not become evident until adolescence or adulthood. Some females with milder forms of the disease who survive to adolescence exhibit ovarian dysfunction. This variant of the disorder is called ovarioleukodystrophy.