Quantity size:

0.2ml

Concentration:

1mg/ml 乙基丙二酸脑病蛋白抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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 乙基丙二酸脑病蛋白抗体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) .乙基丙二酸脑病蛋白抗体 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.

乙基丙二酸脑病蛋白抗体Also known as:

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.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Dog, Pig, Cow, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human ETHE1.

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Predicted Molecular Weight: 26kDa.

乙基丙二酸脑病蛋白抗体Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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