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Both activate AMPK, but through distinct mechanisms: metformin inhibits complex I of the mitochondrial electron transport chain, raising the cellular AMP:ATP ratio and thereby activating AMPK indirectly

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Alternatively, accumulated methylmalonate and methylpropionate, precursors of the cobalamin-dependent synthesis of succinyl-CoA, may be incorporated abnormally into branched-chain fatty acids, resulting in abnormal myelination (88)

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