Article
Betaine supplementation is less effective than methionine restriction in correcting phenotypes of CBS deficient mice.
Journal of inherited metabolic disease - 1 Jan 2016
Gupta Sapna, Wang Liqun, Kruger Warren D
Abstract excerpt
Cystathionine beta synthase (CBS) deficiency is a recessive inborn error of metabolism characterized by elevated serum total homocysteine (tHcy). Betaine supplementation, which can lower tHcy by stimulating homocysteine remethylation to methionine, is often given to CBS deficient patients in combination with other treatments such as methionine restriction and supplemental B-vitamins. However, the effectiveness of...
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