Anti-ETHE1 antibody
| 英文名称 | ETHE1 |
| 中文名称 | 乙基丙二酸脑病蛋白抗体 |
| 别 名 | mitochondrial; Ethe1; ETHE1 protein, mitochondrial precursor; ETHE1_HUMAN; ethylmalonic encephalopathy 1; Ethylmalonic encephalopathy protein 1; hepatoma subtracted clone one; Hepatoma subtracted clone one protein; HSCO; Protein ETHE1; YF13H12. |
DATASHEET
Host:Rabbit
Target Protein:ETHE1
IR:Immunogen Range:191-254/254
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:23474
Swiss Prot:O95571
Source:KLH conjugated synthetic peptide derived from human ETHE1:191-254/254
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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.
Size:100ul
Concentration:1mg/ml
Applications:ELISA(1:5000-10000)
Cross Reactive Species:Human
Mouse
Dog
Pig
Cow
Rabbit
Sheep
.
For research use only. Not intended for diagnostic or therapeutic use.
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