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FIGURE 4 The CBL primarily catalyzes the cleavage of cystathionine, producing homocysteine, pyruvate, and ammonia

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At the renal level, these toxins induce direct cytotoxicity (mitochondrial dysfunction, DNA damage, epigenetic alterations), endothelial dysfunction (via VCAM1, ICAM1, tissue factor), and activation of both innate (macrophages, ILC3s) and adaptive (Th1/Th17/Treg dysregulation, Bcell modulation) immune responses

Altered status of glutathione and its metabolites in cystinotic cells

Determination of free thiols The redox state of GRXs was determined by alkylation of free thiols and subsequent analysis of the mobility of the alkylated protein on non-reducing SDS PAGE 34