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Homocysteine accumulates in the blood when there is inadequate folate and/or vitamin B 12 for effective functioning of the methionine synthase enzyme (see Function)

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Charnas LR, Luciano CA, Dalakas M, Gilliatt RW, Bernardini I, Ishak K, Cwik VA, Fraker D, Brushart TA, Gahl WA (1994) Distal vacuolar myopathy in nephropathic cystinosis

12,21,22 The opening of the mitochondrial permeability transition pore under pathological conditions, such as oxidative stress, calcium overload, or energy depletion, leads to disruption of the mitochondrial membrane potential and the release of proapoptotic factors, ultimately culminating in cell death

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