Ndufs4 knockout mice with isolated complex I deficiency engage a futile adaptive brain response - supplementary data

This study aims to provide insight into the Leigh Syndrome pathomechanism at the sub-brain level. To this end, a comparative proteome analysis was performed on various brain regions of 6 wildtype (WT) and 6 whole body (WB) Ndufs4-/- (WB-KO) mice over two experiments. Samples were collected at six weeks after birth (i.e. ~ one week prior to WB-KO death). For the first experiment samples were collected from a brain slice (BrSl), which contained (parts of) the CC, HC, hypothalamus, thalamus, caudate putamen, and olfactory areas. For the second experiment the analysed brain regions consisted of cerebellum (CB), cerebral cortex (CC), hippocampus (HC), inferior colliculust (IC), and superior colliculus (SC).