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Description
It plays a crucial role in transporting fatty acids into mitochondria to produce adenosine triphosphate (ATD), which is vital for energy production in skeletal muscle and heart

15 At higher doses, Lcarnitine is metabolized to trimethylamine and butyrobetaine

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Harding originally classified dHMN into 7 categories, based on inheritance pattern, age of onset, severity, and distinguishing clinical features (Fig

Trimethyl-lysine residues are the primary precursors for carnitine biosynthesis, which are hydroxylated on the third carbon by the enzyme trimethyl-lysine deoxygenase (TMLD) in mitochondria of liver, kidney, heart, muscle and brain [5]
