Translating m-AAA protease function in mitochondria to hereditary spastic 
paraplegia

Rugarli, E. I.; Langer, T.

doi:10.1016/j.molmed.2006.04.002

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Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia

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Langer, T.
Department Langer - Mitochondrial Proteostasis, Max Planck Institute for Biology of Ageing, Max Planck Society;

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https://www.ncbi.nlm.nih.gov/pubmed/16647881
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Citation

Rugarli, E. I., & Langer, T. (2006). Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia. Trends Mol Med, 12(6), 262-9. doi:10.1016/j.molmed.2006.04.002.

Cite as: https://hdl.handle.net/21.11116/0000-000B-7287-C

Abstract

Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that is characterized by progressive and cell-specific axonal degeneration. An autosomal recessive form of the disease is caused by mutations in paraplegin, which is a conserved subunit of the ubiquitous and ATP-dependent m-AAA protease in mitochondria. The m-AAA protease carries out protein quality control in the inner membrane of the mitochondria, suggesting a pathogenic role of misfolded proteins in HSP. A recent study demonstrates that the m-AAA protease regulates ribosome assembly and translation within mitochondria by controlling proteolytic maturation of a ribosomal subunit. Here, we will discuss implications of the dual role of the m-AAA protease in protein activation and degradation for mitochondrial dysfunction and axonal degeneration.