Trans-sulfuration in Mammals

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Trans-sulfuration in mammals. The methionine-sparing effect of cystine.

1. Hepatic levels of cystathionine synthase and methionine-activating enzyme are significantly lower in rats fed a diet low in methionine and supplemented with cystine than in rats growing at the same rate while maintained on a diet adequate in methionine, with or without cystine supplementation. Cystathionase levels are also decreased, but to a smaller extent. Betaine-homocysteine methyltransf...

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Liver intracellular L-cysteine concentration is maintained after inhibition of the trans-sulfuration pathway by propargylglycine in rats.

To study the fate of L-cysteine and amino acid homeostasis in liver after the inhibition of the trans-sulfuration pathway, rats were treated with propargylglycine (PPG). At 4 h after the administration of PPG, liver cystathionase (EC 4.4.1.1) activity was undetectable, L-cystathionine levels were significantly higher, L-cysteine was unchanged and GSH concentration was significantly lower than v...

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Functional characterization of a methionine gamma-lyase in Arabidopsis and its implication in an alternative to the reverse trans-sulfuration pathway.

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Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury.

Cysteine is considered a nonessential amino acid in mammals as it is synthesized from methionine via trans-sulfuration. However, premature infants or patients with hepatic failure may require dietary cysteine due to a lack of cystathionine gamma-lyase (CTH), a key trans-sulfuration enzyme. Here, we generated CTH-deficient (Cth(-/-)) mice as an animal model of cystathioninemia/cystathioninuria. ...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 1967

ISSN: 0021-9258

DOI: 10.1016/s0021-9258(18)96205-8