Complete sequencing of the 7 mtDNA genes encoding for complex I subunits in frontotemporal lobar degeneration

Abstract

Background: Mitochondria dysfunction and oxidative damage were reported in aging-related neurodegenerative diseases. A possible mechanism involves mitochondrial DNA (mtDNA) alterations impairing mitochondrial respiratory chain function. Contrary to other neurological disorders, frontotemporal lobar degeneration (FTLD) pathophysiology is still poorly understood, and etiology is often unknown. Recently, two mtDNA alterations were reported in a FTLD patient and an association with mtDNA haplogroup was proposed.