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Biochemical abnormalities resulting from accumulation of hydrogen sulfide and its derivative thiosulfate in crucial tissues including liver, brain, and colonic mucosa leading to inhibition of both short-chain acyl-CoA dehydrogenase with consequent elevation of ethylmalonate, C4- and C5-acylcarnitines predominantly in muscle and brain, and cytochrome c oxidase deficiency, blocking mitochondrial respiration and increasing lactic acid [147,148]