Complexome profiling of heart mitochondria from WT and ClpP-/- mice

Mutations in the gene for the mitochondrial matrix protease CLPP can cause human Perrault syndrome, which is characterized by male and female infertility, progressive sensorineural deafness, ataxia and leukoencephalopathy. This gene encodes a peptidase that is conserved since bacteria and localizes to mitochondrial matrix in eukaryotes. To assess the assembly and stability of mitochondrial complexes heart tissue of WT and ClpP-/- mice was dissected and enriched mitochondrial fraction was used for complexome profiling.