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[DOI] [PMC free article] [PubMed] [Google Scholar] 385.Khanna R., Nandy D., Senapati S

Of particular interest, in contrast to the first patients described with GCS deficiency, this patient had no neurologic disease

It has been established that the total pool of intracellular thiols decreases when cysteine metabolism is limited as a result of cysB and cysE gene deletions by 54 and 62% with inhibition of glutathione synthesis ( gshAB deletions) by 56%, and with increased import of cystine (P tet -tcyP ) or slowing the export of cysteine ( eamA ) by 48 and 40%, respectively (Fig

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