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- W2123202217 abstract "The molecular motor dynein and its associated regulatory subunit dynactin have been implicated in several neurodegenerative conditions of the basal ganglia, such as Huntington's disease (HD) and Perry syndrome, an atypical Parkinson-like disease. This pathogenic role has been largely postulated from the existence of mutations in the dynactin subunit p150(Glued). However, dynactin is also able to act independently of dynein, and there is currently no direct evidence linking dynein to basal ganglia degeneration. To provide such evidence, we used here a mouse strain carrying a point mutation in the dynein heavy chain gene that impairs retrograde axonal transport. These mice exhibited motor and behavioural abnormalities including hindlimb clasping, early muscle weakness, incoordination and hyperactivity. In vivo brain imaging using magnetic resonance imaging showed striatal atrophy and lateral ventricle enlargement. In the striatum, altered dopamine signalling, decreased dopamine D1 and D2 receptor binding in positron emission tomography SCAN and prominent astrocytosis were observed, although there was no neuronal loss either in the striatum or substantia nigra. In vitro, dynein mutant striatal neurons displayed strongly impaired neuritic morphology. Altogether, these findings provide a direct genetic evidence for the requirement of dynein for the morphology and function of striatal neurons. Our study supports a role for dynein dysfunction in the pathogenesis of neurodegenerative disorders of the basal ganglia, such as Perry syndrome and HD." @default.
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- W2123202217 date "2010-08-31" @default.
- W2123202217 modified "2023-10-17" @default.
- W2123202217 title "A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons" @default.
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- W2123202217 cites W1530320970 @default.
- W2123202217 cites W1532388773 @default.
- W2123202217 cites W1545569420 @default.
- W2123202217 cites W1651597776 @default.
- W2123202217 cites W1964181591 @default.
- W2123202217 cites W1977649097 @default.
- W2123202217 cites W1978104052 @default.
- W2123202217 cites W1978215017 @default.
- W2123202217 cites W1978999158 @default.
- W2123202217 cites W1984979116 @default.
- W2123202217 cites W1985659221 @default.
- W2123202217 cites W1985813762 @default.
- W2123202217 cites W1989976072 @default.
- W2123202217 cites W1993386867 @default.
- W2123202217 cites W1995764872 @default.
- W2123202217 cites W1996617402 @default.
- W2123202217 cites W1997985916 @default.
- W2123202217 cites W2007706798 @default.
- W2123202217 cites W2009510719 @default.
- W2123202217 cites W2012279551 @default.
- W2123202217 cites W2020887697 @default.
- W2123202217 cites W2021258666 @default.
- W2123202217 cites W2025615154 @default.
- W2123202217 cites W2026750744 @default.
- W2123202217 cites W2029061472 @default.
- W2123202217 cites W2037640340 @default.
- W2123202217 cites W2044024554 @default.
- W2123202217 cites W2044782567 @default.
- W2123202217 cites W2047638794 @default.
- W2123202217 cites W2052970339 @default.
- W2123202217 cites W2053623116 @default.
- W2123202217 cites W2054230322 @default.
- W2123202217 cites W2055022577 @default.
- W2123202217 cites W2055057832 @default.
- W2123202217 cites W2056599855 @default.
- W2123202217 cites W2063815643 @default.
- W2123202217 cites W2065943966 @default.
- W2123202217 cites W2069722515 @default.
- W2123202217 cites W2074189105 @default.
- W2123202217 cites W2075357057 @default.
- W2123202217 cites W2076788657 @default.
- W2123202217 cites W2082229119 @default.
- W2123202217 cites W2086528223 @default.
- W2123202217 cites W2088331424 @default.
- W2123202217 cites W2094612064 @default.
- W2123202217 cites W2095099413 @default.
- W2123202217 cites W2103444316 @default.
- W2123202217 cites W2106117608 @default.
- W2123202217 cites W2110029962 @default.
- W2123202217 cites W2111405228 @default.
- W2123202217 cites W2112969008 @default.
- W2123202217 cites W2117671988 @default.
- W2123202217 cites W2121972502 @default.
- W2123202217 cites W2132498101 @default.
- W2123202217 cites W2135864962 @default.
- W2123202217 cites W2145359342 @default.
- W2123202217 cites W2148937096 @default.
- W2123202217 cites W2155683196 @default.
- W2123202217 cites W2160529952 @default.
- W2123202217 cites W2169478622 @default.
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- W2123202217 doi "https://doi.org/10.1093/hmg/ddq361" @default.
- W2123202217 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3298848" @default.
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- W2123202217 hasPublicationYear "2010" @default.
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