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Transsulfuration pathway Ability for the liver cell to convert methionine to cysteine is important since the liver is the major site of methionine catabolism and the major storage organ for GSH It is absent or insignificant in other GSH-synthesizing systems Markedly impaired or absent in the fetus and newborn infant, cirrhotic patients, and patients with homocystinemia the Km of hepatic methionine adenosyltransferase for ATP is high (2 mM) hypoxic depletion of ATP is more likely to affect GSH synthesis from methionine than cysteine Transsulfuration pathway Methionine and homocysteine are readily interconvertible The transsulfuration pathway converts methionine to cysteine, which is then converted to GSH via the GSH synthetic pathway Methionine can also be resynthesized from homocysteine The control appears to be exerted at the level of homocysteine: when methionine is needed, homocysteine is remethylated by methionine synthase or betaine-homocysteine methyltransferase

Arakawa, H

Biochemical identification and crystal structure of kynurenine formamidase from Drosophila melanogaster

The reaction catalyzed by G6PD is unique to the pathway

Y.EnglundE.HoltonJ