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Mitochondrial dysfunction in protein conformational disorders

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Abstract

Protein aggregation is a hallmark of many neurodegenerative diseases. In Parkinson’s disease protein misfolding of \(\upalpha \)-synuclein involves conformational changes in the protein structure that often results in self-association and aggregation leading to accumulation of \(\upalpha \)-synuclein in neuronal cells. The underlying mechanisms by which aggregations can lead to impaired cellular functions are often not understood. Meanwhile, there is growing evidence that links mitochondrial dysfunction to Parkinson’s disease. As both mitochondria and protein aggregation of \(\upalpha \)-synuclein have been shown to play a major role in Parkinson’s disease, it seems likely that a converging mechanism exists that links the two pathways.

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Authors and Affiliations

  1. Department of Experimental Neurodegeneration, University Medical Center Gottingen, Waldweg 33, Göttingen, Germany

    Daniel Kaganovich

  2. Department of Cell and Developmental Biology, Hebrew University of Jerusalem, Givat Ram, Jerusalem, Israel

    Shlomi Brielle & Daniel Kaganovich

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  1. Shlomi Brielle
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  2. Daniel Kaganovich
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Correspondence to Shlomi Brielle or Daniel Kaganovich.

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Brielle, S., Kaganovich, D. Mitochondrial dysfunction in protein conformational disorders. J Genet 97, 703–713 (2018). https://doi.org/10.1007/s12041-018-0958-0

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  • DOI: https://doi.org/10.1007/s12041-018-0958-0

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