Reference Report

Reference
Citation	Kanuka, H., Kuranaga, E., Hiratou, T., Igaki, T., Nelson, B., Okano, H., Miura, M. (2003). Cytosol-endoplasmic reticulum interplay by Sec61α translocon in polyglutamine-mediated neurotoxicity in Drosophila. Proc. Natl. Acad. Sci. U.S.A. 100(20): 11723--11728. (Export to RIS)
FlyBase ID	FBrf0173066
Publication Type	Research paper
PubMed ID	14504396
PubMed Abstract	Intracellular deposition of aggregated and ubiquitinated proteins is a prominent cytopathological feature of most neurodegenerative disorders frequently correlated with neural cell death. To elucidate mechanisms in neural cell death and degeneration, we characterized the Drosophila ortholog of Sec61alpha (DSec61alpha), a component of the translocon that is involved in both protein import and endoplasmic reticulum-associated degradation. Loss-of-function experiments for DSec61alpha revealed that the translocon contributes to expanded polyglutamine-mediated neuronal toxicity, likely resulting from proteasome inhibition and leading to accumulation of ubiquitinated proteins. Taken together, proteasome inhibition by expanded polyglutamine tracts may lead to the accumulation of toxic undegraded proteins normally transported by the Sec61alpha translocon.
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Language of Publication	English
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Publication Type	Journal
Abbreviation	Proc. Natl. Acad. Sci. U.S.A.
Title	Proceedings of the National Academy of Sciences of the United States of America
Publication Year	1915-
ISBN/ISSN	0027-8424
Data from Reference
Alleles (17)
	Avic\GFP^RN3.Scer\UAS Hsap\ATXN3^{Q17.Scer\UAS.T:Avic\GFP-EGFP} Hsap\ATXN3^{Q60.Scer\UAS.T:Avic\GFP-EGFP} Hsap\ATXN3^{Q150.Scer\UAS.T:Avic\GFP-EGFP} Hsap\ATXN3^{tr.Q78.Scer\UAS.T:Ivir\HA1} Hsap\HTT^GMR.Q120 Prosβ6¹ Scer\GAL4^Act5C.PHb Scer\GAL4^GMR.PF Scer\GAL4^Rh3.PP Sec61α^dsRNA.cKa Sec61α^{dsRNA.Scer\UAS} Sec61α^EP2567 Sec61α^k04917 Sec61α^P560ex25 Sec61α^{Scer\UAS.cKa.T:Zzzz\FLAG} T:Avic\GFP^EGFP
Constructs (8)
	P{GAL4-ninaE.GMR} P{GMR-HTT.Q120} P{Rh3-GAL4} P{UAS-Hsap\MJD.Q17} P{UAS-Hsap\MJD.Q60} P{UAS-Hsap\MJD.Q150} P{UAS-Hsap\MJD.tr-Q78} P{UAS-Sec61α.FLAG}
Genes (11)
	Avic\GFP Hsap\ATXN3 Hsap\HTT Prosβ6 Scer\GAL4 Sec61α T:Avic\GFP T:Ivir\HA1 T:Zzzz\FLAG Ubi-p5E Ubi-p63E
Insertions (2)
	P{EP}Sec61α^EP2567 P{lacW}Sec61α^k04917