Reference Report

Reference
Citation	Lam, Y.C., Bowman, A.B., Jafar-Nejad, P., Lim, J., Richman, R., Fryer, J.D., Hyun, E.D., Duvick, L.A., Orr, H.T., Botas, J., Zoghbi, H.Y. (2006). ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell 127(7): 1335--1347. (Export to RIS)
FlyBase ID	FBrf0193611
Publication Type	Research paper
PubMed ID	17190598
PubMed Abstract	Spinocerebellar ataxia type 1 (SCA1) is one of several neurodegenerative diseases caused by expansion of a polyglutamine tract in the disease protein, in this case, ATAXIN-1 (ATXN1). A key question in the field is whether neurotoxicity is mediated by aberrant, novel interactions with the expanded protein or whether its wild-type functions are augmented to a deleterious degree. We examined soluble protein complexes from mouse cerebellum and found that the majority of wild-type and expanded ATXN1 assembles into large stable complexes containing the transcriptional repressor Capicua. ATXN1 directly binds Capicua and modulates Capicua repressor activity in Drosophila and mammalian cells, and its loss decreases the steady-state level of Capicua. Interestingly, the S776A mutation, which abrogates the neurotoxicity of expanded ATXN1, substantially reduces the association of mutant ATXN1 with Capicua in vivo. These data provide insight into the function of ATXN1 and suggest that SCA1 neuropathology depends on native, not novel, protein interactions.
DOI	10.1016/j.cell.2006.11.038
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Secondary IDs
Language of Publication	English
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Publication Type	Journal
Abbreviation	Cell
Title	Cell
Publication Year	1974-
ISBN/ISSN	0092-8674
Data from Reference
Alleles (10)
	aos^W11 Avic\GFP^Scer\UAS.cUa cic^D49 cic^Scer\UAS.cLa Ecol\lacZ^aos-W11 Hsap\ATXN1^82Q.Scer\UAS Scer\GAL4^C5 Scer\GAL4^GMR.PF w^+mC Zzzz\CAG^{127Q.Scer\UAS.T:Ivir\HA1}
Constructs (5)
	P{GAL4-ninaE.GMR} P{UAS-cic.L} P{UAS-GFP.U} P{UAS-Hsap\ATX1.82Q} P{UAS-Zzzz\CAG.127Q}
Genes (8)
	aos Avic\GFP cic Ecol\lacZ Hsap\ATXN1 Scer\GAL4 w Zzzz\CAG
Insertions (5)
	P{GawB}C5^C5 P{lwB}aos^W11 P{UAS-Hsap\ATX1.30Q}F6 P{UAS-Hsap\ATX1.82Q}M6 P{UAS-Hsap\ATX1.82Q}N35Y
Natural transposons (1)
	P-element