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The Role of Chaperones and Co-Chaperones in Retinal Degenerative Diseases

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

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

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Molecular chaperones play an important role in normal retina function and are implicated in several forms of retinal dystrophy. The retina is a specialized part of the CNS that presents a fascinating paradigm to investigate molecular chaperone function. Mutations in several photoreceptor proteins lead to protein misfolding mediated neurodegeneration. The best characterized of these are mutations in the molecular light sensor, rhodopsin, which cause autosomal dominant retinitis pigmentosa. Rhodopsin biogenesis may require chaperones and rhodopsin misfolding involves molecular chaperones in quality control and the cellular response to protein aggregation. Furthermore, the specialization of components of chaperone machinery to photoreceptor specific roles has been revealed by the identification of mutations in putative chaperone proteins that cause inherited retinal dysfunction and degeneration. These putative chaperones are involved in several important cellular pathways and further illuminate the essential and diverse roles of molecular chaperones in the nervous system

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Authors and Affiliations

  1. Division of Molecular and Cellular Neuroscience, Institute of Ophthalmology, University College London, London, UK

    Maria Kosmaoglou, Tatiana V. Novoselova & Michael E. Cheetham

Authors
  1. Maria Kosmaoglou
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  2. Tatiana V. Novoselova
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  3. Michael E. Cheetham
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Editor information

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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© 2008 Springer Science+Business Media B.V.

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Kosmaoglou, M., Novoselova, T.V., Cheetham, M.E. (2008). The Role of Chaperones and Co-Chaperones in Retinal Degenerative Diseases. 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_5

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