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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
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ETHE1 Antibody / Persulfide dioxygenase ETHE1, mitochondrial Antibody detects ETHE1, a mitochondrial sulfur dioxygenase involved in the metabolism of hydrogen sulfide-derived compounds. Hydrogen sulfide is produced during sulfur-containing amino acid metabolism and can act in signaling at controlled concentrations, but excessive accumulation interferes with mitochondrial respiration. ETHE1 catalyzes the oxygen-dependent oxidation of persulfide sulfur to sulfite, forming part of the pathway that disposes of sulfide-derived sulfur. The enzyme contains a non-heme iron center important for its catalytic activity.
Mitochondrial sulfide oxidation involves several cooperating components. Sulfide:quinone oxidoreductase initiates oxidation and transfers electrons into the ubiquinone pool, while downstream reactions process sulfur intermediates. ETHE1 acts on persulfide substrates generated within this network. The pathway links sulfur handling with respiratory-chain activity and cellular redox balance. ETHE1 is not the same enzyme as sulfite oxidase, which acts at a later stage of sulfur metabolism. Keeping these steps distinct helps explain why deficiencies in different enzymes can produce different biochemical patterns. The Persulfide dioxygenase ETHE1, mitochondrial Antibody designation refers to this same molecular target.
Biallelic pathogenic variants in ETHE1 cause ethylmalonic encephalopathy, a severe metabolic disorder associated with impaired sulfide detoxification. Accumulated sulfide can inhibit cytochrome c oxidase, helping explain the relationship between sulfur metabolism and mitochondrial dysfunction. The disease association provides strong evidence for the physiological importance of ETHE1 activity. NSJ Bioreagents includes ETHE1 among mitochondrial metabolic targets. An ETHE1 Antibody provides a way to investigate the biological role of ETHE1 and the molecular processes associated with this target.
Explore related research targets in our Metabolism Antibody / Metabolism Antibodies collection.
Users should optimize the working dilution of the ETHE1 Antibody / Persulfide dioxygenase ETHE1, mitochondrial Antibody for their specific experimental setup.
The ETHE1 Antibody was generated using e. coli-derived human ETHE1 recombinant protein (amino acids D42-A254).
Following reconstitution, the ETHE1 Antibody can be kept at 4oC for one month. Aliquot for extended storage at -20oC for up to six months, avoiding repeated freezing and thawing.
ETHE1 antibody, Persulfide dioxygenase ETHE1, mitochondrial antibody, Persulfide dioxygenase ETHE1 antibody, mitochondrial antibody, ETHE1 persulfide dioxygenase antibody, ethylmalonic encephalopathy 1 antibody, HSCO antibody, Protein ETHE1 antibody, YF13H12 antibody
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