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Cystinosis, since being first described in 1903 [4], to discovering the genetic defect in the CTNS gene almost a century later in 1998 [5], is a very complex disease

Since GSH biosynthesis is an energy-dependent process, we postulated that in patients with ETC defects GSH status becomes compromised, leading to further loss of ETC activity

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Main Points From Todays Episode 1

Boehm-Cagan et al., 2014) but not in other studies (Veeraraghavalu et al., 2013

Estimation of serum protein level Protein estimations were carried out according to the method of Lowry et al.36 Under alkaline condition the divalent copper ion form a complex with peptide bond in which it is reduced to a monovalent ion