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Data from MARCH/MARCH-ON, a clinical trial of maralixibat for individuals with PFIC, demonstrated reductions in sBA

cK1-1 and cK1-2 were folded into cK1-1f and cK1-2f, respectively, using the glutathione/glutathione disulfide redox system 46

Premature aging and disturbed methylation are components of DS [99]

This dual-hit mechanism - combining enhanced oxidative stress with compromised redox regulation - not only establishes a pathological link between diabetic metabolic dysregulation and tissue damage, but also suggests the therapeutic potential of modulating BCAAs metabolism to restore redox homeostasis