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Role of HspB1 and HspB8 in Hereditary Peripheral Neuropathies: Beyond the Chaperone Function

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Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection

Part of the book series: Heat Shock Proteins ((HESP,volume 3))

abstract

Within the last 10 years, mutations in genes encoding the small heat shock proteins (also called HspB) HspB1, HspB4, HspB5 and HspB8 have been associated with neurological and muscular disorders. In particular, HspB1 and HspB8 mutations result in hereditary peripheral neuropathies, which primarily affect motor and/or sensory peripheral neurons. Due to their extremely long axons, peripheral neurons are particularly dependent on an efficient vesicular trafficking and axonal transport, whose defects have been directly linked to the development of hereditary peripheral neuropathies. Genetic analyses generated new insights into the molecular pathways involved in hereditary peripheral neuropathies, which include not only vesicular trafficking but also protein quality control, protein degradation and RNA processing. This review summarizes the current understanding of HspB implication in motor diseases, starting with a general picture of HspB functions followed by a description of new emerging roles for HspB1 and HspB8 in axonal transport, protein sorting and degradation

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Authors and Affiliations

  1. Department of Radiation and Stress Cell Biology, University Medical Center Groningen, Groningen, The Netherlands

    Serena Carra

  2. Centre de recherche en cancérologie de l’Université Laval L’Hôtel-Dieu de Québec, Québec, Canada

    Jacques Landry

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  1. Serena Carra
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  2. Jacques Landry
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Editors and Affiliations

  1. Effie and Wofford Cain Centennial Endowed Chair in Clinical Pathology, Chief, Division of Investigative Pathology, Scott & White Memorial Hospital and Clinic and The Texas A&M Health Science Center College of Medicine, Temple, Texas, U.S.A

    Alexzander A.A. Asea

  2. Department of Biological Sciences, Center for the Neurobiology of Stress, University of Toronto at Scarborough, Toronto, ON, Canada

    Ian R. Brown

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Carra, S., Landry, J. (2008). Role of HspB1 and HspB8 in Hereditary Peripheral Neuropathies: Beyond the Chaperone Function. In: Asea, A.A., Brown, I.R. (eds) Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection. Heat Shock Proteins, vol 3. Springer, Dordrecht. https://doi.org/10.1007/978-1-4020-8231-3_7

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