Inhibited ATP production with a simultaneously unaffected ATPase function has been reported in some neuropathy, ataxia, retinitis pigmentosa (NARP), and Leigh syndrome mutations, possibly because of disturbances in proton import into the mitochondrial matrix or defects in the coupling of proton flow through the F0 subunit and ATP synthesis in the F1 subunit [64]. Here, DNAH8 is linked to NARP syndrome.