Rabbit ETHE1 Polyclonal Antibody | anti-ETHE1 antibody

ETHE1 Polyclonal Antibody

Cat. Num. AAA3008382

• Gene Names: ETHE1; HSCO; YF13H12
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ETHE1; Polyclonal Antibody; ETHE1 Polyclonal Antibody; HSCO; Sulfur dioxygenase ETHE1; anti-ETHE1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ETHE1 polyclonal antibody detects endogenous levels of ETHE1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 254

• Applicable Applications for anti-ETHE1 antibody: Western Blot (WB)
• Application Notes: WB: 1:200-1:1000
• Immunogen: Recombinant protein of human ETHE1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot analysis of extracts of various cells, using ETHE1 antibody.)

Related Product Information for anti-ETHE1 antibody

ETHE1 (ethylmalonic encephalopathy 1), also known as HSCO (hepatoma subtracted clone one protein), is a 254 amino acid protein belonging to the metallo-beta-lactamase superfamily and glyoxalase II family. Localizing to the cytoplasm, nucleus and mitochondrion matrix, ETHE1 is ubiquitously expressed and may function in sulfide catabolism. ETHE1 binds two zinc ions per subunit and interacts directly with RELA, preventing its localization to the nucleus thus leading to suppressed p53-induced apoptosis. The gene encoding ETHE1 maps to human chromosome 19q13.31. Mutations to this gene result in ethylmalonic encephalopathy, an infantile metabolic disorder characterized by high levels of ethylmalonic acid, neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, and death within the first decade of life.

NCBI and Uniprot Product Information

• NCBI GI #: 41327741
• NCBI GeneID: 23474
• NCBI Accession #: NP_055112.2
• NCBI GenBank Nucleotide #: NP_055112.2
• UniProt Accession #: O95571
• Molecular Weight: ~ 28kDa
• NCBI Official Full Name: persulfide dioxygenase ETHE1, mitochondrial isoform 1
• NCBI Official Synonym Full Names: ETHE1 persulfide dioxygenase
• NCBI Official Symbol: ETHE1
• NCBI Official Synonym Symbols: HSCO; YF13H12
• NCBI Protein Information: persulfide dioxygenase ETHE1, mitochondrial
• UniProt Protein Name: Persulfide dioxygenase ETHE1, mitochondrial
• Protein Family: Persulfide dioxygenase
• UniProt Gene Name: ETHE1
• UniProt Synonym Gene Names: HSCO
• UniProt Entry Name: ETHE1_HUMAN

NCBI Description

This gene encodes a member of the metallo beta-lactamase family of iron-containing proteins involved in the mitochondrial sulfide oxidation pathway. The encoded protein catalyzes the oxidation of a persulfide substrate to sulfite. Certain mutations in this gene cause ethylmalonic encephalopathy, an infantile metabolic disorder affecting the brain, gastrointestinal tract and peripheral vessels. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Mar 2016]

Uniprot Description

ETHE1: 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. 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. Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.

Protein type: Hydrolase; Mitochondrial; EC 1.13.11.18

Chromosomal Location of Human Ortholog: 19q13.31

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; cytoplasm

Molecular Function: iron ion binding; sulfur dioxygenase activity

Biological Process: glutathione metabolic process; sulfur amino acid metabolic process; sulfur amino acid catabolic process

Disease: Encephalopathy, Ethylmalonic