Talk:Glutathione synthetase deficiency

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[edit] glutathione/cysteine

Has anyone seen the orphanet webpage for glutathione synthase deficiency? <http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1852094> It says this : "N-Acetylcysteine used to be recommended because it may protect cells from oxidative stress. However, cysteine has been shown to accumulate in tissues of patients with glutathione synthetase deficiency – at least in cultured fibroblasts [24]. Since cysteine is known to be neurotoxic in excessive amounts [32], treatment with N-acetylcysteine should not be recommended for patients with glutathione synthetase deficiency as this may increase the intracellular cysteine levels even more."

Is this true? This goes against decades of knowledge about this disorder. It says that these people with GSD shouldn't 

take cysteine b/c it accumulates in their tissues and is neurotoxic. Is this true... I've never heard this before. —Preceding unsigned comment added by Interestedperson (talk • contribs) 15:47, 1 May 2008 (UTC)