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Once synthesized, it is distributed through the bloodstream and becomes highly concentrated in tissues that demand massive amounts of continuous energy, specifically the skeletal muscles and the myocardium (heart muscle), which contain over 95% of the body's total carnitine stores

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Direct comparison of ML226 treatment to lentiviral ABHD11 sgRNA depletion demonstrated that both pharmacological and genetic inhibition of ABHD11 reduced functional lipoylation on the E2 subunit of the OGDHc (dihydrolipoamide succinyltransferase (DLST), referred to as the OGDHc-E2) in preference to the E2 subunit of PDHc (dihydrolipoamide acetyltransferase (DLAT), referred to as the PDHc-E2