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Specifications
- Antibody type:Primary
- Antigen name:Ethylmalonic encephalopathy protein 1
- Clonality:Polyclonal
- Gene ID:23474
- Host:Rabbit
- Isotype:IgG
- Reactivity:Human,Mouse
- Antigen symbol:ETHE1
- Conjugation:HRP (Horseradish Peroxidase)
- ImmunoChemistry:Yes
- Size:100 µL
- Western blot:Yes
- Cross adsorption:No
- Form:liquid
- Antigen synonyms:HSCO|Hepatoma subtracted clone one protein
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% Gentamicin. Store at 4°C for 12 months.
- Storage temperature:Store at –20 °C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- Cat. no.:10465-760
- Supplier No.:BS-7610R-HRP
Specifications
About this item
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.
Recommended Dilutions: Western Blot: 1:100-1000; IHC-P: 1:100-500
Type: Primary
Antigen: ETHE1
Clonality: Polyclonal
Clone:
Conjugation: HRP (Horseradish Peroxidase)
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse