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L-Cystine is a dimeric form of cysteine that is formed by the covalent oxidative linkage of two cysteine residues.1 It accumulates in the lysosomes of patients with cystinosis, an autosomal recessive lysosomal storage disorder, and is associated with renal Fanconi syndrome and loss of glomerular function.2
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1. Techniques to monitor disulfide bond formation and the reduction potential of cysteine–cystine couples in vitro and in vivo. Oxidative folding of proteins: Basic principles, cellular regulation and engineering 34-51 (2018).
2. Cystinosis: A review. Orphanet J. Rare Dis. 11, 47 (2016).