| Overview |
| bs-7610R |
| ETHE1 Polyclonal Antibody |
| ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC) |
| Human, Mouse, Dog, Cow, Sheep, Pig, Rabbit |
| Specifications |
| Unconjugated |
| Rabbit |
| KLH conjugated synthetic peptide derived from human E1 |
| 191-254/254 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Target |
| 23474 |
| Cytoplasm, Nucleus |
| 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. |
| 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. |
| Application Dilution |
| ELISA |
1:500-1000 |
| IHC-P |
1:200-400 |
| IHC-F |
1:100-500 |
| IF(IHC-P) |
1:50-200 |
| IF(IHC-F) |
1:50-200 |
| IF(ICC) |
1:50-200 |
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