Abstract：
:SCA1, a fatal neurodegenerative disorder, is caused by a CAG expansion encoding a polyglutamine stretch in the protein ATXN1. We used RNA sequencing to profile cerebellar gene expression in Pcp2-ATXN1[82Q] mice with ataxia and progressive pathology and Pcp2-ATXN1[30Q]D776 animals having ataxia in absence of Purkinje cell progressive pathology. Weighted Gene Coexpression Network Analysis of the cerebellar expression data revealed two gene networks that significantly correlated with disease and have an expression profile correlating with disease progression in ATXN1[82Q] Purkinje cells. The Magenta Module provides a signature of suppressed transcriptional programs reflecting disease progression in Purkinje cells, while the Lt Yellow Module reflects transcriptional programs activated in response to disease in Purkinje cells as well as other cerebellar cell types. Furthermore, we found that upregulation of cholecystokinin (Cck) and subsequent interaction with the Cck1 receptor likely underlies the lack of progressive Purkinje cell pathology in Pcp2-ATXN1[30Q]D776 mice.
journal_name
Neuronjournal_title
Neuronauthors
Ingram M,Wozniak EAL,Duvick L,Yang R,Bergmann P,Carson R,O'Callaghan B,Zoghbi HY,Henzler C,Orr HTdoi
10.1016/j.neuron.2016.02.011subject
Has Abstractpub_date
2016-03-16 00:00:00pages
1194-1207issue
6eissn
0896-6273issn
1097-4199journal_volume
89pub_type
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