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Trends Mol Med. 2006 Jun;12(6):262-9. Epub 2006 May 2.

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

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Istituto Nazionale Neurologico C. Besta, Division of Biochemistry and Genetics, 20126 Milan, Italy.


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.

[Indexed for MEDLINE]

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