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These conditions are characterized by very high (FAODs, ischaemia) or modestly increased (type 2 diabetes mellitus) tissue and blood levels of LCACs

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Cystine is the least soluble of the common amino acids, and its aberrant accumulation in the cytosol can be highly cytotoxic because it induces disulfide stress, forcing cells to quickly reduce cystine to cysteine, which is much more soluble 11