Reference Report

Reference
Citation	Mizutani, A., Wang, L., Rajan, H., Vig, P.J.S., Alaynick, W.A., Thaler, J.P., Tsai, C.C. (2005). Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1. EMBO J. 24(18): 3339--3351. (Export to RIS)
FlyBase ID	FBrf0187513
Publication Type	Research paper
PubMed ID	16121196
PubMed Abstract	Ataxin-1 is a neurodegenerative disorder protein whose glutamine-repeat expanded form causes spinocerebellar ataxia type 1 (SCA1) in humans and exerts cytotoxicity in Drosophila and mouse. We report here that the cytotoxicity caused by ataxin-1 is modulated by association with a related protein, Brother of ataxin-1 (Boat). Boat and ataxin-1 share a conserved AXH (ataxin-1 and HMG-box protein 1) domain, which is essential for both proteins' interactions with the transcriptional corepressor SMRT and its Drosophila homolog, SMRTER. The Boat-ataxin-1 interaction is mediated through multiple regions in both proteins, including a newly identified NBA (N-terminal region of Boat and ataxin-1) domain. We investigated the physiological relevance of the Boat-ataxin-1 interaction in Drosophila and discovered that a mutant ataxin-1-mediated eye defect is suppressed by ataxin-1's association with Boat. Correspondingly, in transgenic SCA1 mouse, Boat expression is greatly reduced in Purkinje cells, the primary targets of SCA1. Our study thus establishes that Boat is an in vivo binding partner of ataxin-1 whose altered expression in Purkinje cells may contribute to their degeneration in SCA1 animals.
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Language of Publication	English
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Parent Publication
Publication Type	Journal
Abbreviation	EMBO J.
Title	The EMBO Journal
Publication Year	1982-
ISBN/ISSN	0261-4189
Data from Reference
Alleles (8)
	Avic\GFP^Scer\UAS.cUa Hsap\ATXN1^{0Q.Scer\UAS.T:Zzzz\FLAG} Hsap\ATXN1^82.Scer\UAS Hsap\ATXN1L^{Scer\UAS.T:Ivir\HA1,T:Zzzz\nls1} Hsap\ATXN1L^{Scer\UAS.T:Zzzz\FLAG} Hsap\ATXN3^{tr.Q78.Scer\UAS.T:Ivir\HA1} Scer\GAL4^GMR.PF Zzzz\CAG^{127Q.Scer\UAS.T:Ivir\HA1}
Constructs (8)
	P{GAL4-ninaE.GMR} P{UAS-GFP.U} P{UAS-Hsap\ATX1.0Q.FLAG} P{UAS-Hsap\ATX1.82} P{UAS-Hsap\Boat.FLAG} P{UAS-Hsap\Boat.HA.nls} P{UAS-Hsap\MJD.tr-Q78} P{UAS-Zzzz\CAG.127Q}
Genes (11)
	Avic\GFP futsch Hsap\ATXN1 Hsap\ATXN1L Hsap\ATXN3 Scer\GAL4 Smr T:Ivir\HA1 T:Zzzz\FLAG T:Zzzz\nls1 Zzzz\CAG
Natural transposons (1)
	P-element