Journal
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Volume 109, Issue 39, Pages 15918-15923Publisher
NATL ACAD SCIENCES
DOI: 10.1073/pnas.1205102109
Keywords
animal model; PARK7; neuritic beading; neuronal death; neuroinflammation
Categories
Funding
- Parkinson Society Canada
- Canadian Institutes of Health Research
- Centres of Excellence in Neurodegeneration (COEN)
- Heart and Stroke Foundation of Ontario (HSFO)
- Neuroscience Canada/Krembil Foundation
- Parkinson's Disease Foundation
- The Michael J. Fox Foundation for Parkinson's Research
- Parkinson Research Consortium (PRC)
- Canadian Stroke Network
- Heart and Stroke Foundation of Canada (HSFC) for Stroke Recovery
- World Class University Program through National Research Foundation of Korea
- Ministry of Education, Science, and Technology, South Korea [R31-2008-000-20004-0]
- HSFO
- HSFC Focus on Stroke Award
- Canadian Institutes of Health Research (CIHR) Training Program in Neurodegenerative Lipidomics Supplement Scholarship
- The PRC Toth Family Fellowship in Parkinson's Research
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DJ-1 mutations cause autosomal recessive early-onset Parkinson disease (PD). We report a model of PD pathology: the DJ1-C57 mouse. A subset of DJ-1-nullizygous mice, when fully backcrossed to a C57BL/6J background, display dramatic early-onset unilateral loss of dopaminergic (DA) neurons in their substantia nigra pars compacta, progressing to bilateral degeneration of the nigrostriatal axis with aging. In addition, these mice exhibit age-dependent bilateral degeneration at the locus ceruleus nucleus and display mild motor behavior deficits at aged time points. These findings effectively recapitulate the early stages of PD. Therefore, the DJ1-C57 mouse provides a tool to study the preclinical aspects of neurodegeneration. Importantly, by exome sequencing, we identify candidate modifying genes that segregate with the phenotype, providing potentially critical clues into how certain genes may influence the penetrance of DJ-1-related degeneration in mice.
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