乙基丙二酸脑病蛋白抗体
规格：1mg/1ml
英文名： ETHE1
别名： mitochondrial; Ethe1; ETHE1 protein, mitochondrial precursor; ETHE1_HUMAN; ethylmalonic encephalopathy 1; Ethylmalonic encephalopathy protein 1; hepatoma subtracted clone one; Hepatoma subtracted clon
分子量： 26kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ETHE1
交叉反应：Human, Mouse, Dog, Pig, Cow, Rabbit, Sheep,
细胞定位：细胞核 细胞浆
乙基丙二酸脑病蛋白抗体产品介绍：background: 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. Function: 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乙基丙二酸脑病蛋白抗体 preventing nuclear localization of RELA. Subunit: Interacts with RELA. Subcellular Location: Cytoplasm. Nucleus. Mitochondrion matrix. Note=According to PubMed:12398897, it is cytoplasmic and nuclear. According to PubMed:14732903, it is found in the mitochondrial matrix. Tissue Specificity: Ubiquitously expressed. DISEASE: Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) [MIM:602473]. EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the乙基丙二酸脑病蛋白抗体 first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria. Similarity: Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family. Gene ID: 23474 Database links: Entrez Gene: 23474 Human Entrez Gene: 66071 Mouse Entrez Gene: 292710 Rat Omim: 608451 Human SwissProt: O95571 Human SwissProt: Q9DCM0 Mouse Unigene: 7486 Human Unigene: 29553 Mouse Unigene: 14691 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
乙基丙二酸脑病蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
研究领域：细胞生物 信号转导 细胞凋亡
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid