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Basis for a therapeutic role for carnitine in propionic acidemia. Due to a metabolic block, propionyl-coenzyme A accumulates in the mitochondrial matrix. An alternative pathway exists to remove this toxic intermediate as propionyl-carnitine, a harmless by-product, but there is insufficient endogenous carnitine available. Supplemental L-carnitine enhances this pathway, thereby releasing coenzyme-A for its vital functions.
