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In cystinosis, mutations in the gene for cystinosin give rise to the lack of a functional lysosomal cystine transporter (43)

In contrast, a recent study provided histopathological evidence of neurodegenerative progression that is related to KD in a transgenic mice model with impaired mitochondrial DNA repair, although there is an increase in mitochondrial biogenesis and antioxidant signatures (45)

1c, Supplementary Figs 1 and 2)

These findings confirmed that the compound maintained stability over a shorter distance with the residue GLN 228 at a 100-ns simulation

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