期刊
DEVELOPMENTAL NEUROSCIENCE
卷 27, 期 5, 页码 333-348出版社
KARGER
DOI: 10.1159/000086713
关键词
ATP7A; Menkes disease; olfactory system; olfactory receptor neurons; neurodevelopment; regeneration; process outgrowth; synaptogenesis
资金
- NIDDK NIH HHS [DK32949] Funding Source: Medline
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [R37DK032949, R56DK032949, R01DK032949] Funding Source: NIH RePORTER
Menkes disease (MD) is a neurodegenerative disorder caused by mutation of the copper transporter ATP7A. While several enzymes expressed in mature neurons require copper, MD neurodegenerative changes cannot be explained by known requirements forATP7A in neuronal development. To investigate additional roles for ATP7A during development, we characterized its pattern of expression using the olfactory system as a neurodevelopmental model. ATP7A expression in neurons was developmentally regulated rather than constitutively. Initially expressed in the cell bodies of developing neurons, ATP7A protein later shifted to extending axons, peaking prior to synaptogenesis. Similarly, after injury-stimulated neurogenesis, ATP7A expression increased in neurons and axons preceding synaptogenesis. Interestingly, copper-transport-deficient ATP7A still exhibits axonal localization. These results support a role forATP7A in axon extension, which may contribute to the severe neurodegeneration characteristic of MD. Copyright (c) 2005 S. Karger AG, Basel.
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