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Protein quality control in mitochondria and neurodegeneration in hereditary spastic paraplegia

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Mitochondrial Function and Biogenesis

Part of the book series: Topics in Current Genetics ((TCG,volume 8))

Abstract

Impaired proteolysis and protein folding leading to the accumulation of protein aggregates has been implicated in a series of neurodegenerative disorders. Research in this field has emphasised the important role of protein quality control systems consisting of molecular chaperones, proteases and ancillary factors in disease mechanisms. Mitochondrial dysfunction has recently been linked to neurodegeneration by the identification of disease-causing mutations in components of the highly conserved and ubiquitous organellar quality control system. These include a membrane-bound ATP-dependent AAA protease as well as the mitochondrial chaperonin Hsp60. A functional impairment of either component causes axonal degeneration in hereditary spastic paraplegia (HSP). This review summarises our current knowledge on mitochondrial protein quality control and its implications for the pathogenesis of HSP.

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Authors and Affiliations

  1. Research Unit for Molecular Medicine, Aarhus University Hospital, Aarhus, Denmark

    Peter Bross

  2. Telethon Institute of Genetics and Medicine (TIGEM), Naples, Italy

    Elena I. Rugarli

  3. Human Molecular Genetics Unit, Department of Neuroscience, San Raffaele Scientific Institute, Milan, Italy

    Giorgio Casari

  4. Institute for Genetics and Center for Molecular Medicine (CMMC), University of Cologne, Zülpicher Str. 47, 50674 Cologne, Germany

    Thomas Langer

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  1. Peter Bross
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  2. Elena I. Rugarli
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  3. Giorgio Casari
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  4. Thomas Langer
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© 2004 Springer-Verlag Berlin/Heidelberg

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Bross, P., Rugarli, E.I., Casari, G., Langer, T. (2004). Protein quality control in mitochondria and neurodegeneration in hereditary spastic paraplegia. In: Mitochondrial Function and Biogenesis. Topics in Current Genetics, vol 8. Springer, Berlin, Heidelberg. https://doi.org/10.1007/b95865

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  • DOI: https://doi.org/10.1007/b95865

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-21489-2

  • Online ISBN: 978-3-540-40895-6

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