Reference Report

Reference
Citation	Nisoli, I., Chauvin, J.P., Napoletano, F., Calamita, P., Zanin, V., Fanto, M., Charroux, B. (2010). Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy. Cell Death Differ. 17(10): 1577--1587. (Export to RIS)
FlyBase ID	FBrf0212035
Publication Type	Research paper
PubMed ID	20339376
PubMed Abstract	Polyglutamine pathologies are neurodegenerative diseases that manifest both general polyglutamine toxicity and mutant protein-specific effects. Dentatorubral-pallidoluysian Atrophy (DRPLA) is one of these disorders caused by mutations in the Atrophin-1 protein. We have generated several models for DRPLA in Drosophila and analysed the mechanisms of cellular and organism toxicity. Our genetic and ultrastructural analysis of neurodegeneration suggests that autophagy may have a role in cellular degeneration when polyglutamine proteins are overexpressed in neuronal and glial cells. Clearance of autophagic organelles is blocked at the lysosomal level after correct fusion between autophagosomes and lysosomes. This leads to accumulation of autofluorescent pigments and proteinaceous residues usually degraded by the autophagy-lysosome system. Under these circumstances, further pharmacological and genetic induction of autophagy does not rescue neurodegeneration by polyglutamine Atrophins, in contrast to many other neurodegenerative conditions. Our data thus provide a crucial insight into the specific mechanism of a polyglutamine disease and reveal important differences in the role of autophagy with respect to other diseases of the same family.
DOI	10.1038/cdd.2010.31
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Language of Publication	English
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Publication Type	Journal
Abbreviation	Cell Death Differ.
Title	Cell Death and Differentiation
Publication Year	1994-
ISBN/ISSN	1350-9047
Data from Reference
Alleles (28)
	Atg1^GS10797 Atg1^Δ3D Atg5^{dsRNA.Scer\UAS} Atg8a^{Scer\UAS.T:Avic\GFP} DnaJ-1^Scer\UAS.cKa Gug³⁵ Gug^{66QC.Scer\UAS} Gug^{75QN.Scer\UAS} Gug^j5A3 Gug^Scer\UAS.cCa Hsap\ATN1^65Q.Scer\UAS Hsap\ATN1^{N917.65Q.Scer\UAS} Hsap\ATN1^wt.Scer\UAS Hsap\ATN1^ΔC.Scer\UAS Hsap\ATN1^{ΔC.Ubi.T:Ivir\HA1} Hsap\HTT^{Q93.ex1p.Scer\UAS} Rheb^Scer\UAS.cUa Scer\GAL4^Act5C.PU Scer\GAL4^Eaat1.PR Scer\GAL4^elav.PU Scer\GAL4^en.PU Scer\GAL4^GMR.PU Scer\GAL4^ninaE.PT Scer\GAL4^ptc-559.1 Scer\GAL4^repo.PU Scer\GAL4^tub.PU Scer\GAL80^ts.αTub84B Tor^TED.Scer\UAS
Constructs (23)
	P{Act5C-GAL4.U} P{Eaat1-GAL4.R} P{elav-GAL4.U} P{en-GAL4.U} P{GMR-GAL4.U} P{repo-GAL4.U} P{rh1-GAL4} P{tub-GAL4.U} P{tubP-GAL80^ts} P{UAS-Atg5.IR} P{UAS-Atg8a.GFP} P{UAS-ATN1.65Q} P{UAS-ATN1.N917.65Q} P{UAS-ATN1.wt} P{UAS-ATN1.ΔC} P{UAS-Atro.66QC} P{UAS-Atro.75QN} P{UAS-Atro.C} P{UAS-DnaJ-1.K} P{UAS-HTT.Q93.ex1p} P{UAS-Rheb.U} P{UAS-Tor.TED} P{Ubi-ATN1.ΔC.HA}
Genes (13)
	Atg1 Atg5 Atg8a DnaJ-1 Gug Hsap\ATN1 Hsap\HTT Rheb Scer\GAL4 Scer\GAL80 T:Ivir\HA1 Tor Tsc1
Insertions (2)
	P{GawB}ptc^559.1 P{GSV6}Atg1^GS10797
Natural transposons (1)
	P-element