Mitochondrial Complex V Deficiency, Mitochondrial Type 1, also known as mitochondrial complex v deficiency mitochondrial type 1, is related to mitochondrial disease and leigh syndrome. An important gene associated with Mitochondrial Complex V Deficiency, Mitochondrial Type 1 is MT-ATP6 (Mitochondrially Encoded ATP Synthase Membrane Subunit 6), and among its related pathways/superpathways is Mitochondrial complex III assembly. Affiliated tissues include heart and skin.