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Clinical and molecular characterization of 6 children with glutamate-cysteine ligase deficiency causing hemolytic anemia

Development of kidney disease is associated with the enzyme myeloperoxidase (MPO), abundant in neutrophils catalyzing the transformation of hydrogen peroxide (H 2 O 2 ) to hypochlorous acid (HOCl), a powerful oxidizing cytotoxic agent, according to the reaction HOCl has been found to be responsible for renal dysfunction among obese children

Hypertonic saline does not affect lung tissue levels of GSH or SCN Since hypertonic saline does increase the ELF levels of both GSH and SCN in WT mice, the lung tissue GSH and SCN levels were measured to determine whether the intracellular levels are also increased

The sample concentration was based on an 8 point standard curve made from known concentrations of NaSCN

Graham, B