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Mitochondrial encephalomyopathy is a complex group of inherited disease with multisystem involvement that have mitochondrial respiratory chain dysfunction. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the most common well-defined mitochondrial encephalomyopathies, with a frequency of 1:6000 [1,2]. Although the most common mutation in MELAS is in the MT-TL1 gene encoding tRNALeu(UUR), known as adenine to guanine transition at nucleotide 3243 (m.3243A>G) [3,4], multiple other mutations in MELAS have been reported in mitochondrial DNA (mtDNA) and nuclear DNA (nDNA) such as mutations of m.10158T>C [5], m.10191T>C [6,7], m.9553G>A [8] in mtDNA and mutations of POLG [4] in nDNA.