ArticleFASEB journal : official publication of the Federation of American Societies for Experimental Biology2014
Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways.
Article in FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 25 citations in OpenAlex.
- Dietary Taurine Supplementation Improves Sperm Quality and Modulates Seminal Plasma Metabolism in Heat-Stressed Dairy Goat Bucks.Animals : an open access journal from MDPI · 2026Article
- Transsulfuration Pathway Products and HInternational journal of molecular sciences · 2025Review
- GOT2: a moonlighting enzyme at the crossroads of cancer metabolism and theranostics.Frontiers in immunology · 2025Review
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- Spotlight on GOT2 in Cancer Metabolism.OncoTargets and therapy · 2023Review
- Article
- Cysteine Aminotransferase (CAT): A Pivotal Sponsor in Metabolic Remodeling and an Ally of 3-Mercaptopyruvate Sulfurtransferase (MST) in Cancer.Molecules (Basel, Switzerland) · 2020Review
- Metabolic phenotypes of standard and cold-stored platelets.Transfusion · 2020Article
- Cystathionine β-synthase deficiency: Of mice and men.Molecular genetics and metabolism · 2017Review
- Downregulation of hepatic betaine:homocysteine methyltransferase (BHMT) expression in taurine-deficient mice is reversed by taurine supplementation in vivo.Amino acids · 2016Article
- Taurine Transporter Gene Expression in Mononuclear Blood Cells of Type 1 Diabetes Patients.Journal of diabetes research · 2016Article
- Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
Cystathionine β-synthase-deficient homocystinuria (HCU) is a serious life-threatening inborn error of sulfur metabolism with poorly understood pathogenic mechanisms. We investigated the effect of HCU on hepatic cysteine oxidation in a transgenic mouse model of the disease. Cysteine dioxygenase (CDO) protein levels were 90% repressed without any change in mRNA levels. Cysteinesulfinic acid decarboxylase (CSAD) was induced at both the mRNA (8-fold) and protein (15-fold) levels. Cysteine supplementation normalized CDO protein levels without reversing the induction of CSAD. Regulatory changes in CDO and CSAD expression were proportional to homocysteine elevation, indicating a possible threshold effect. Hepatic and blood taurine levels in HCU animals were decreased by 21 and 35%, respectively, and normalized by cysteine supplementation. Expression of the cytoplasmic (GOT1) and mitochondrial (GOT2) isoforms of glutamic-oxaloacetic transaminase were repressed in HCU animals by 86 and 30%, respectively. HCU induced regulatory changes in CSAD, CDO, and GOT1 expression were normalized by taurine supplementation, indicating that cysteine is not the only sulfur compound that regulates hepatic cysteine oxidation. Collectively, our results indicate that HCU induces significant alterations of sulfur metabolism with the potential to contribute to pathogenesis and that cysteine and taurine have the potential to serve as adjunctive treatments in this disease.
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