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Reduced hepatic cystathionine gamma-lyase activity

MedGen UID:
1841972
•Concept ID:
C5826777
•
Finding
Synonym: Reduced hepatic cystathionase activity
 
HPO: HP:0034929

Definition

Activity of cystathionine gamma-lyase in the liver below the lower limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReduced hepatic cystathionine gamma-lyase activity

Recent clinical studies

Therapy

Hepatic hydrogen sulfide levels are reduced in mouse model of Hutchinson-Gilford progeria syndrome.
Wilkie SE, Marcu DE, Carter RN, Morton NM, Gonzalo S, Selman C
Aging (Albany NY) 2023 Jun 23;15(12):5266-5278. doi: 10.18632/aging.204835. PMID: 37354210Free PMC Article
SREBP-1c impairs ULK1 sulfhydration-mediated autophagic flux to promote hepatic steatosis in high-fat-diet-fed mice.
Nguyen TTP, Kim DY, Lee YG, Lee YS, Truong XT, Lee JH, Song DK, Kwon TK, Park SH, Jung CH, Moon C, Osborne TF, Im SS, Jeon TI
Mol Cell 2021 Sep 16;81(18):3820-3832.e7. Epub 2021 Jul 6 doi: 10.1016/j.molcel.2021.06.003. PMID: 34233158
Dietary methionine restriction reduces hepatic steatosis and oxidative stress in high-fat-fed mice by promoting H(2)S production.
Yang Y , Wang Y , Sun J , Zhang J , Guo H , Shi Y , Cheng X , Tang X , Le G
Food Funct 2019 Jan 22;10(1):61-77. doi: 10.1039/c8fo01629a. PMID: 30534793
Chronic aspartame intake causes changes in the trans-sulphuration pathway, glutathione depletion and liver damage in mice.
Finamor I, Pérez S, Bressan CA, Brenner CE, Rius-Pérez S, Brittes PC, Cheiran G, Rocha MI, da Veiga M, Sastre J, Pavanato MA
Redox Biol 2017 Apr;11:701-707. Epub 2017 Feb 1 doi: 10.1016/j.redox.2017.01.019. PMID: 28187322Free PMC Article

Clinical prediction guides

Cystathionine gamma-lyase/H(2)S system suppresses hepatic acetyl-CoA accumulation and nonalcoholic fatty liver disease in mice.
Ali A, Zhang Y, Fu M, Pei Y, Wu L, Wang R, Yang G
Life Sci 2020 Jul 1;252:117661. Epub 2020 Apr 16 doi: 10.1016/j.lfs.2020.117661. PMID: 32305523

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