期刊
FASEB JOURNAL
卷 28, 期 2, 页码 781-790出版社
FEDERATION AMER SOC EXP BIOL
DOI: 10.1096/fj.13-240770
关键词
osteoporosis; alopecia; homocysteine; metabolism; inborn errors
资金
- Monell Chemical Senses Center under a grant from the Pennsylvania Department of Health
- Hempling Foundation for Homocystinuria Research, the U.S. National Institutes of Health [CA06927, R01GM098772]
Cystathionine -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.Gupta, S., Melnyk, S.B., Kruger, W.D. Cystathionine -synthase-deficient mice thrive on a low-methionine diet.
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