Proteome of WT and CLPP-null Podospora Anserina

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 compare and validate mouse and human findings, the filamentous fungus Podospora anserina was cultured and protein extracts were analyzed.