Role of the ubiquitin–proteasome system in nervous system function and disease: using C. elegans as a dissecting tool

In addition to its central roles in protein quality control, regulation of cell cycle, intracellular signaling, DNA damage response and transcription regulation, the ubiquitin–proteasome system (UPS) plays specific roles in the nervous system, where it contributes to precise connectivity through dev...

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Bibliographic Details
Published in:Cellular and molecular life sciences : CMLS 2012-08, Vol.69 (16), p.2691-2715
Main Authors: Baptista, Márcio S., Duarte, Carlos B., Maciel, Patrícia
Format: Article
Language:English
Subjects:
Animals
Biochemistry
Biomedical and Life Sciences
Biomedicine
Caenorhabditis elegans
Caenorhabditis elegans - genetics
Caenorhabditis elegans - metabolism
Cell Biology
Eukaryotes
Life Sciences
Nematodes
Nervous System Physiological Phenomena
Neurodegenerative Diseases - metabolism
Neurological disorders
Proteasome Endopeptidase Complex - metabolism
Proteins
Quality control
Review
Ubiquitin - metabolism
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Summary:In addition to its central roles in protein quality control, regulation of cell cycle, intracellular signaling, DNA damage response and transcription regulation, the ubiquitin–proteasome system (UPS) plays specific roles in the nervous system, where it contributes to precise connectivity through development, and later assures functionality by regulating a wide spectrum of neuron-specific cellular processes. Aberrations in this system have been implicated in the etiology of neurodevelopmental and neurodegenerative diseases. In this review, we provide an updated view on the UPS and highlight recent findings concerning its role in normal and diseased nervous systems. We discuss the advantages of the model organism Caenorhabditis elegans as a tool to unravel the major unsolved questions concerning this biochemical pathway and its involvement in nervous system function and dysfunction, and expose the new possibilities, using state-of-the-art techniques, to assess UPS function using this model system.
ISSN:1420-682X
1420-9071
DOI:10.1007/s00018-012-0946-0