Complexome profiling of brain mitochondrial membranes from Clpp-/- mouse

The autosomal recessive Perrault syndrome with juvenile ovarian failure, progressive sensorineural deafness, ataxia and leukoencephalopathy can be caused by loss of function mutations in CLPP. This gene encodes a peptidase that is conserved since bacteria and localizes to mitochondrial matrix in eukaryotes. Here, assembly and stability of mitochondrial complexes from adult Clpp-/- mouse brains were analyzed by Complexome profiling.