ε-Trimethyllysine hydroxylase (TMLH) is a homodimer enzyme located in the submitochondrial matrix. TMLH is a ferrous iron and 2-ketoglutarate dependent oxygenase that catalyze the stereospecific oxidation of ε-trimethyllysine (TML) to β-hydroxy-TML (HTML) that is a first step in the L-carnitine biosynthesis pathway. The inhibition of the TMLH is proposed to have the similar and more potent effect on the energy metabolism processes than meldonium (clinically used anti-ischemia drug). Thus, it will become possible to reduce significantly the concentrations of the L-carnitine and to shift the energy metabolism from the β-oxidation of long-chain fatty acids to glycolysis.