ETHE1, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Affinity Purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ETHE1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This is a rabbit polyclonal antibody against ETHE1. It was validated on Western Blot

Target Description: This gene encodes a sulfur dioxygenase that localizes within the mitochondrial matrix. The enzyme functions in sulfide catabolism. Mutations in this gene result in ethylmalonic encephalopathy.

Polyclonal; Drugs and Drug Metabolism; Cell Biology; Mitochondria;

Western Blot (WB)

WB Suggested Anti-ETHE1 Antibody Titration: 0.2-1 ug/ml
ELISA Titer: 1:62500
Positive Control: COLO205 cell lysateETHE1 is supported by BioGPS gene expression data to be expressed in COLO205

28kDa

ETHE1 persulfide dioxygenase

persulfide dioxygenase ETHE1, mitochondrial

Persulfide dioxygenase ETHE1, mitochondrial

HSCO  [Similar Products]

ETHE1_HUMAN

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]

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

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