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Quoted phrase not found in phrase index: "Sulfide quinone oxidoreductase deficiency"
Page 1
Human ultrarare genetic disorders of sulfur metabolism demonstrate redundancies in H2S homeostasis.
Kožich V, Schwahn BC, Sokolová J, Křížková M, Ditroi T, Krijt J, Khalil Y, Křížek T, Vaculíková-Fantlová T, Stibůrková B, Mills P, Clayton P, Barvíková K, Blessing H, Sykut-Cegielska J, Dionisi-Vici C, Gasperini S, García-Cazorla Á, Haack TB, Honzík T, Ješina P, Kuster A, Laugwitz L, Martinelli D, Porta F, Santer R, Schwarz G, Nagy P. Kožich V, et al. Redox Biol. 2022 Dec;58:102517. doi: 10.1016/j.redox.2022.102517. Epub 2022 Oct 18. Redox Biol. 2022. PMID: 36306676 Free PMC article.
We analyzed sulfur compounds (including bioavailable sulfide) in 37 untreated or insufficiently treated patients with seven ultrarare enzyme deficiencies and compared them to 63 controls. Surprisingly, we observed that patients with severe deficiency in cysta …
We analyzed sulfur compounds (including bioavailable sulfide) in 37 untreated or insufficiently treated patients with seven ultrarare enzyme …
Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway.
Ziosi M, Di Meo I, Kleiner G, Gao XH, Barca E, Sanchez-Quintero MJ, Tadesse S, Jiang H, Qiao C, Rodenburg RJ, Scalais E, Schuelke M, Willard B, Hatzoglou M, Tiranti V, Quinzii CM. Ziosi M, et al. EMBO Mol Med. 2017 Jan;9(1):96-111. doi: 10.15252/emmm.201606356. EMBO Mol Med. 2017. PMID: 27856618 Free PMC article.
Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. ...These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss …
Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of the hydrogen sulfide …
Hydrogen sulfide as an anti-calcification stratagem in human aortic valve: Altered biogenesis and mitochondrial metabolism of H(2)S lead to H(2)S deficiency in calcific aortic valve disease.
Combi Z, Potor L, Nagy P, Sikura KÉ, Ditrói T, Jurányi EP, Galambos K, Szerafin T, Gergely P, Whiteman M, Torregrossa R, Ding Y, Beke L, Hendrik Z, Méhes G, Balla G, Balla J. Combi Z, et al. Redox Biol. 2023 Apr;60:102629. doi: 10.1016/j.redox.2023.102629. Epub 2023 Feb 8. Redox Biol. 2023. PMID: 36780769 Free PMC article.
The expression of mitochondrial enzymes involved in H(2)S catabolism including sulfide quinone oxidoreductase (SQR), the key enzyme in mitochondrial H(2)S oxidation, persulfide dioxygenase (ETHE1), sulfite oxidase (SO) and thiosulfate sulfurtransferase (TST) …
The expression of mitochondrial enzymes involved in H(2)S catabolism including sulfide quinone oxidoreductase (SQR), th …
Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism.
González-García P, Hidalgo-Gutiérrez A, Mascaraque C, Barriocanal-Casado E, Bakkali M, Ziosi M, Abdihankyzy UB, Sánchez-Hernández S, Escames G, Prokisch H, Martín F, Quinzii CM, López LC. González-García P, et al. Hum Mol Genet. 2020 Nov 25;29(19):3296-3311. doi: 10.1093/hmg/ddaa214. Hum Mol Genet. 2020. PMID: 32975579 Free PMC article.
Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10 (CoQ10) deficiency. This finding leads us to hypothesize that the therapeutic effects of CoQ10, frequently administered to patients with primary or secondary mitochondrial dysfunction, might be due …
Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10 (CoQ10) deficiency. This finding leads us to hypothesize …
Proteomics reveals that redox regulation is disrupted in patients with ethylmalonic encephalopathy.
Palmfeldt J, Vang S, Stenbroen V, Pavlou E, Baycheva M, Buchal G, Monavari AA, Augoustides-Savvopoulou P, Mandel H, Gregersen N. Palmfeldt J, et al. J Proteome Res. 2011 May 6;10(5):2389-96. doi: 10.1021/pr101218d. Epub 2011 Mar 28. J Proteome Res. 2011. PMID: 21410200
Deficiency of the sulfide metabolizing protein ETHE1 is the cause of ethylmalonic encephalopathy (EE), an inherited and severe metabolic disorder. ...Two proteins involved in pathways of detoxification and oxidative/reductive stress were underrepresented in EE patient samp …
Deficiency of the sulfide metabolizing protein ETHE1 is the cause of ethylmalonic encephalopathy (EE), an inherited and severe metabo …