ArticleFASEB journal : official publication of the Federation of American Societies for Experimental Biology2014
Cystathionine β-synthase-deficient mice thrive on a low-methionine diet.
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 18 papers.
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18 citing papers in PubMed, 32 citations in OpenAlex.
- Dietary Shift Leads to Venous Thrombosis-Induced Congestive Liver Failure in CBS-Deficient Mice.Journal of inherited metabolic disease · 2025Article
- Article
- The association of fasting plasma thiol fractions with body fat compartments, biomarker profile, and adipose tissue gene expression.Amino acids · 2023Article
- Recent therapeutic approaches to cystathionine beta-synthase-deficient homocystinuria.British journal of pharmacology · 2023Review
- Long-term functional correction of cystathionine β-synthase deficiency in mice by adeno-associated viral gene therapy.Journal of inherited metabolic disease · 2021Article
- Analysis of differential neonatal lethality in cystathionine β-synthase deficient mouse models using metabolic profiling.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2019Article
- Treatment of Cystathionine β-Synthase Deficiency in Mice Using a Minicircle-Based Naked DNA Vector.Human gene therapy · 2019Article
- Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria.Molecular therapy : the journal of the American Society of Gene Therapy · 2018Article
- Cystathionine β-synthase deficiency: Of mice and men.Molecular genetics and metabolism · 2017Review
- Enzyme replacement with PEGylated cystathionine β-synthase ameliorates homocystinuria in murine model.The Journal of clinical investigation · 2016Article
- Exploring the Lean Phenotype of Glutathione-Depleted Mice: Thiol, Amino Acid and Fatty Acid Profiles.PloS one · 2016Article
- The effect of dietary modulation of sulfur amino acids on cystathionine β synthase-deficient mice.Annals of the New York Academy of Sciences · 2016Review
- Betaine supplementation is less effective than methionine restriction in correcting phenotypes of CBS deficient mice.Journal of inherited metabolic disease · 2016Article
- Emerging role of hydrogen sulfide in hypertension and related cardiovascular diseases.British journal of pharmacology · 2015Review
- Article
- Body composition in gene knockouts of sulfur amino acid-metabolizing enzymes.Mammalian genome : official journal of the International Mammalian Genome Society · 2014Review
- Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2014Article
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3 authors at 2 institutions in 1 country.
Funding
Abstract
Cystathionine β-synthase (CBS) deficiency is a recessive inborn error of metabolism characterized by elevated serum total homocysteine (tHcy). Previously, our laboratory developed a mouse model of CBS deficiency, TgI278T Cbs(-)/(-) (abbreviated as Cbs(-/-)), characterized by low weight, low adiposity, decreased Scd-1 expression, facial alopecia, and osteoporosis. To determine the potential benefit of a methionine-restricted diet (MRD), we fed Cbs(-/-) and Cbs(+/-) control mice either an MRD or a regular diet (RD) from weaning till 240 d of age. Cbs(-/-) mice fed the MRD had a 77% decrease in tHcy, 28% increase in weight, 130% increase in fat mass, 82% increase in Scd-1 expression, and 10.6% increase in bone density and entirely lacked the alopecia phenotype observed in age-matched Cbs(-/-) mice fed the RD. At the end of the study, Cbs(-/-) mice fed the MRD were phenotypically indistinguishable from Cbs(+/-) mice fed the RD. Notably, whereas the MRD diet was highly beneficial to Cbs(-/-) mice, it had nearly opposite effect on Cbs(+/-) mice. These studies show that a low-methionine diet can correct the phenotypic consequences of loss of CBS and provide a striking example of how genotype and diet can interact to influence phenotype in mammals.
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