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| Status |
Public on Mar 01, 2011 |
| Title |
Polyglutamine Atrophin provokes neurodegeneration in Drosophila |
| Organism |
Drosophila melanogaster |
| Experiment type |
Expression profiling by array
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| Summary |
Dentatorubral-pallidoluysian Atrophy (DRPLA) is a human polyQ disease caused by the expansion of a CAG strech in the atrophin-1 (at-1) gene. In all vertebrates, a second atrophin gene (at-2) is present and it encodes a related protein void of polyQ tracks. In D.melanogaster there is one conserved Atrophin (Atro) gene, ubiquitously expressed, which contains all functional domains of vertebrate Atrophins, including two polyQ stretches. To understand to what extent transcriptional alterations cause neurodegeneration and are linked to the normal functions of Atrophin, we performed a genome wide transcriptional profiling in our Drosophila models, focusing on primary events that precede neurodegeneration.
We have found that polyQ Atro downregulates fat, which protects from neurodegeneration and Atrophin toxicity trhough the Hippo kinase cascade.
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| Overall design |
Neurodegeneration progression caused by poly glutamine expansions of the Atro gene is measured by mRNA expression profiles in 4 D.melanogaster genotypes: in wild-type animals (controls), animals constitutively expressing the wild-type Atro transgene, animals constitutively expressing the polyQ expanded Atro transgenes A66QC and A75QN. The expressions of the Atro genes are under a GMR-Gal4 driver repressed by a Gal80 universal repressor sensitive to temperature. When flies are raised at 18 C, the transgenes are not expressed, but their expression is switched on when they are shifted to the ageing protocol at 29 C at time point day 0. The neurodegeneration is assessed after 2 and 14 days post-high-temperature shift.
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| Contributor(s) |
Fanto M, Blanc E |
| Citation(s) |
21278706 |
| Submission date |
Dec 21, 2010 |
| Last update date |
Aug 28, 2018 |
| Contact name |
Eric Blanc |
| E-mail(s) |
eric.blanc@kcl.ac.uk
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| Phone |
0207 848 6532
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| Organization name |
King's College London
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| Department |
MRC Centre for Developmental Neurobiology
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| Street address |
New Hunt's House
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| City |
London |
| ZIP/Postal code |
SE1 1UL |
| Country |
United Kingdom |
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| Platforms (1) |
| GPL1322 |
[Drosophila_2] Affymetrix Drosophila Genome 2.0 Array |
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| Samples (36)
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| GSM644357 |
Genotype: Control, Day: 2, Repl: 1 |
| GSM644358 |
Genotype: Control, Day: 2, Repl: 2 |
| GSM644359 |
Genotype: Control, Day: 2, Repl: 3 |
| GSM644360 |
Genotype: Control, Day: 14, Repl: 1 |
| GSM644361 |
Genotype: Control, Day: 14, Repl: 2 |
| GSM644362 |
Genotype: Control, Day: 14, Repl: 3 |
| GSM644363 |
Genotype: Atro, Day: 0, Repl: 1 |
| GSM644364 |
Genotype: Atro, Day: 0, Repl: 2 |
| GSM644365 |
Genotype: Atro, Day: 0, Repl: 3 |
| GSM644366 |
Genotype: Atro, Day: 2, Repl: 1 |
| GSM644367 |
Genotype: Atro, Day: 2, Repl: 2 |
| GSM644368 |
Genotype: Atro, Day: 2, Repl: 3 |
| GSM644369 |
Genotype: Atro, Day: 14, Repl: 1 |
| GSM644370 |
Genotype: Atro, Day: 14, Repl: 2 |
| GSM644371 |
Genotype: Atro, Day: 14, Repl: 3 |
| GSM644372 |
Genotype: PolyQ Atro A66QC, Day: 0, Repl: 1 |
| GSM644373 |
Genotype: PolyQ Atro A66QC, Day: 0, Repl: 2 |
| GSM644374 |
Genotype: PolyQ Atro A66QC, Day: 0, Repl: 3 |
| GSM644375 |
Genotype: PolyQ Atro A66QC, Day: 2, Repl: 1 |
| GSM644376 |
Genotype: PolyQ Atro A66QC, Day: 2, Repl: 2 |
| GSM644377 |
Genotype: PolyQ Atro A66QC, Day: 2, Repl: 3 |
| GSM644378 |
Genotype: PolyQ Atro A66QC, Day: 14, Repl: 1 |
| GSM644379 |
Genotype: PolyQ Atro A66QC, Day: 14, Repl: 2 |
| GSM644380 |
Genotype: PolyQ Atro A66QC, Day: 14, Repl: 3 |
| GSM644381 |
Genotype: PolyQ Atro A75QN, Day: 0, Repl: 1 |
| GSM644382 |
Genotype: PolyQ Atro A75QN, Day: 0, Repl: 2 |
| GSM644383 |
Genotype: PolyQ Atro A75QN, Day: 0, Repl: 3 |
| GSM644384 |
Genotype: PolyQ Atro A75QN, Day: 2, Repl: 1 |
| GSM644385 |
Genotype: PolyQ Atro A75QN, Day: 2, Repl: 2 |
| GSM644386 |
Genotype: PolyQ Atro A75QN, Day: 2, Repl: 3 |
| GSM644387 |
Genotype: PolyQ Atro A75QN, Day: 14, Repl: 1 |
| GSM644388 |
Genotype: PolyQ Atro A75QN, Day: 14, Repl: 2 |
| GSM644389 |
Genotype: PolyQ Atro A75QN, Day: 14, Repl: 3 |
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| Relations |
| BioProject |
PRJNA135075 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE26246_RAW.tar |
113.4 Mb |
(http)(custom) |
TAR (of CEL) |
| Processed data included within Sample table |
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