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The Gad Mouse: A Window Into UPS-Related Neurodegeneration and the Function of the Function of the Deubiquitinating Enzyme Uch-L1

  • Chapter
The Proteasome in Neurodegeneration

Abstract

The role of UCH-L1 in neuronal function is becoming more apparent with the use of refined cellular and molecular approaches and several in vitro and in vivo model systems. Although the I93M-UCH-L1 mutant and S18Y-UCH-L1 variant are clearly involved in the pathogenesis of PD, our understanding of the underlying molecular mechanisms of UCH-L1 function is still limited. Further investigation of the relationship between neurodegeneration and UCH-L1 modifications should help to elucidate the mechanisms of pathogenesis in PD and AD and may suggest novel targets for therapeutic intervention.

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Author information

Authors and Affiliations

  1. Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan

    Dr. Jungkee Kwon & Dr. Keiji Wada

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  1. Dr. Jungkee Kwon
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  2. Dr. Keiji Wada
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Editors and Affiliations

  1. Institute of Biomedical Research of the Academy of Athens, Athens, Greece

    Leonidas Stefanis

  2. Sanders-Brown Center on Aging Department of Anatomy and Neurobiology, University of Kentucky, KY, USA

    Jeffrey N. Keller

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Kwon, J., Wada, K. (2006). The Gad Mouse: A Window Into UPS-Related Neurodegeneration and the Function of the Function of the Deubiquitinating Enzyme Uch-L1. In: Stefanis, L., Keller, J.N. (eds) The Proteasome in Neurodegeneration. Springer, Boston, MA. https://doi.org/10.1007/0-387-28500-8_11

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