Cystathionine-synthase-deficient patients do not use the transamination pathway of methionine to reduce hypermethioninemia and homocystinemia☆
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Long-term betaine therapy in a murine model of cystathionine beta-synthase deficient homocystinuria: Decreased efficacy over time reveals a significant threshold effect between elevated homocysteine and thrombotic risk
2012, Molecular Genetics and MetabolismCitation Excerpt :Consequently, those cells that lack transsulfuration will have some capacity for dealing with excess methionine. Methionine and Hcy levels could conceivably be reduced by the transamination of methionine but previous work has concluded that this is not a significant mechanism in HCU [32]. Hcy and methionine can be extruded into the extracellular space where they end up in the circulation and are subsequently excreted in the urine.
Gender differences in methionine accumulation and metabolism in freshly isolated mouse hepatocytes: Potential roles in toxicity
2009, Toxicology and Applied PharmacologyPotential roles of flavin-containing monooxygenases in sulfoxidation reactions of L-methionine, N-acetyl-L-methionine and peptides containing L-methionine
2005, Biochimica et Biophysica Acta - Proteins and Proteomics
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This study was supported by a grant from the Netherlands Organization for Scientific Research (Medigon, NWO).
Copyright © 1989 Published by Elsevier Inc.