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A3243G mitochondrial tRNA leu (UUR) point mutation causes mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome, the most common mitochondrial DNA (mtDNA) disorder, and is also found in patients with maternally inherited diabetes and deafness syndrome (MIDD). To correlate disease manifestations with mutational burden, it is necessary to measure the percentage of A3243G mtDNA mutations.