Abstract：
:Spinocerebellar ataxia type 1 (SCA1) is one of nine inherited, typically adult onset, polyglutamine neurodegenerative diseases. To examine whether development impacts SCA1, we used a conditional transgenic mouse model of SCA1 to delay the postnatal expression of mutant ATXN1 until after completion of cerebellar development. Delayed postnatal expression of mutant ATXN1 led to a substantial reduction in severity of disease in adults in comparison with early postnatal gene expression. This was linked to a destabilization of RORalpha, a transcription factor critical for cerebellar development. In SCA1 mice, there was a depletion of RORalpha and a reduction in expression of genes controlled by RORalpha. Partial loss of RORalpha enhanced mutant ATXN1 pathogenicity. Additionally, evidence points to the existence of a complex containing ATXN1, RORalpha, and the RORalpha coactivator Tip60. These studies indicate RORalpha and Tip60 have a role in SCA1 and suggest a mechanism by which compromising cerebellar development contributes to severity of neurodegeneration in an adult.
journal_name
Celljournal_title
Cellauthors
Serra HG,Duvick L,Zu T,Carlson K,Stevens S,Jorgensen N,Lysholm A,Burright E,Zoghbi HY,Clark HB,Andresen JM,Orr HTdoi
10.1016/j.cell.2006.09.036subject
Has Abstractpub_date
2006-11-17 00:00:00pages
697-708issue
4eissn
0092-8674issn
1097-4172pii
S0092-8674(06)01296-7journal_volume
127pub_type
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