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333, 19 (2003) , A
Check whether the SSA concentration is 1% or less at the time of measurement
GSH + amino acid -Glu-amino acid +CysH-Gly (amino group transfer) GSH + H2O Glu +CysH-Gly (hydrolysis) GSH + GSH -Glu-GSH +CysH-Gly (amino group transfer) 3
This is often accompanied by an improved clinical presentation and more favorable laboratory parameters
Developmental delay is universally associated with pyruvate dehyrogenase deficiency (Sofou et al, 2017)
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