Reaction mixture II for each sample was collected as follows: 1160 l of 0.1-M Tris-HCl buffer with 0.34-mM EDTA (pH 8.5) were sequentially added to 108 l of each supernatant containing residual GSH, and then at the same intervals, 9 l of a solution of 5,5-dithiobis(2-nitrobenzoic acid) or Ellmans reagent (4 mg/ml of absolute methanol) was added to each sample (in the same sequence of samples)
Njalsson R, Carlsson K, Winkler A, Larsson A, Norgren S: Diagnostics in patients with glutathione synthetase deficiency but without mutations in the exons of the GSS gene
doi: 10.1210/jc.2019-00637
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