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The progressive accumulation of dysfunctional mitochondria is implicated in aging and in common diseases of the elderly. To oppose this occurrence, organisms employ a variety of strategies, including the selective degradation of oxidatively damaged and misfolded mitochondrial proteins. Genetic studies in yeast indicate that the  A TPase  A ssociated with diverse cellular  A ctivities (AAA + ) family of mitochondrial proteases account for a substantial fraction of this protein degradation, but their metazoan counterparts have been little studied, despite the fact that mutations in the genes encoding these proteases cause a variety of human diseases. To begin to explore the biological roles of the metazoan mitochondrial AAA +  protease family, we have created a CRISPR/Cas9 allele of the  Drosophila  homolog of  SPG7 , which encodes an inner membrane-localized AAA +  protease known as paraplegin.  Drosophila SPG7  mutants exhibited shortened lifespan, progressive locomotor defects, sensitivity to chemical and environmental stress, and muscular and neuronal degeneration. Ultrastructural examination of photoreceptor neurons indicated that the neurodegenerative phenotype of  SPG7  mutants initiates at the synaptic terminal. A variety of mitochondrial defects accompanied the degenerative phenotypes of  SPG7  mutants, including altered axonal transport of mitochondria, accumulation of electron-dense material in the matrix of flight muscle mitochondria, reduced activities of respiratory chain complexes I and II, and severely swollen and dysmorphic mitochondria in the synaptic terminals of photoreceptors.  Drosophila SPG7  mutants recapitulate key features of human diseases caused by mutations in  SPG7 , and thus provide a foundation for the identification of  Drosophila  paraplegin substrates and strategies that could be used to ameliorate the symptoms of these diseases.

cell death and disease

Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration