产品名称

ETHE1抗体

英文名称

ETHE1

产品规格

50ul  100ul 200ul 

产品货号

GOY-01K2854

产品分类

抗体 / 一抗

交叉反应

Human, Mouse, Dog, Pig, Cow, Rabbit, Sheep

保存条件

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

标记

Unconjugated

抗体来源

Rabbit

抗体类型

Monoclonal 

研究领域

细胞生物 信号转导 细胞凋亡

蛋白细胞定位

细胞核,细胞浆

纯化方法

affinity purified by Protein A

性状

Liquid

浓度

1mg/ml

储存液

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

发货地

上海

背景资料

Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.