Journal
CELL REPORTS
Volume 30, Issue 3, Pages 642-+Publisher
CELL PRESS
DOI: 10.1016/j.celrep.2019.12.069
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Funding
- NIH [RO1 NS096352, RO1 NS090914, RO1 NS094754, F31 NRSA 106777-01]
- Duke Chancellor's Discovery Award
- Ruth K. Broad Biomedical Research Foundation's Research Award for Graduate Students
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Huntington's disease (HD) is caused by an autosomal dominant polyglutamine expansion mutation of Huntingtin (HTT). HD patients suffer from progressive motor, cognitive, and psychiatric impairments, along with significant degeneration of the striatal projection neurons (SPNs) of the striatum. HD is widely accepted to be caused by a toxic gain-of-function of mutant HTT. However, whether loss of HTT function, because of dominant-negative effects of the mutant protein, plays a role in HD and whether HTT is required for SPN health and function are not known. Here, we delete Htt from specific subpopulations of SPNs using the Cre-Lox system and find that SPNs require HTT for motor regulation, synaptic development, cell health, and survival during aging. Our results suggest that loss of HTT function in SPNs could play a critical role in HD pathogenesis.
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