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Ethylmalonic encephalopathy

Proteins (3)

ProteinSynonymsTaxonomy
Thiosulfate sulfurtransferaseEC 2.8.1.1; RhodaneseHomo sapiens (human)
Persulfide dioxygenase ETHE1, mitochondrialEC 1.13.11.18; Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1Homo sapiens (human)
Sulfite oxidase, mitochondrialEC 1.8.3.1Homo sapiens (human)

Compounds (7)

CompoundDescription
Hydrogen SulfideA flammable, poisonous gas with a characteristic odor of rotten eggs. It is used in the manufacture of chemicals, in metallurgy, and as an analytical reagent. (From Merck Index, 11th ed)
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
Pyruvic AcidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
ThiosulfatesInorganic salts of thiosulfuric acid possessing the general formula R2S2O3.
SulfitesInorganic salts of sulfurous acid.
coenzyme Q10Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins