ETHE1 antibody (AA 191-254) (AbBy Fluor® 594)

Catalog No. ABIN2812956

Product Details anti-ETHE1 Antibody (AbBy Fluor® 594)

Target: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Binding Specificity: AA 191-254

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This ETHE1 antibody is conjugated to AbBy Fluor® 594

Application: Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human,Mouse,Dog,Cow,Sheep,Pig,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human E1

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for ETHE1

Target: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Alternative Name: ETHE1 (ETHE1 Products)

Synonyms: zgc:85680 antibody, HSCO antibody, YF13H12 antibody, 0610025L15Rik antibody, Hsco antibody, ETHE1, persulfide dioxygenase antibody, ethylmalonic encephalopathy 1 antibody, Ethe1 antibody, ethe1 antibody, ETHE1 antibody

Background:

Synonyms: 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.

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.

Gene ID: 23474