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Citation
Napoletano, F., Occhi, S., Calamita, P., Volpi, V., Blanc, E., Charroux, B., Royet, J., Fanto, M. (2011). Polyglutamine Atrophin provokes neurodegeneration in Drosophila by repressing fat.  EMBO J. 30(5): 945--958.
FlyBase ID
FBrf0213147
Publication Type
Research paper
Abstract
Large alterations in transcription accompany neurodegeneration in polyglutamine (polyQ) diseases. These pathologies manifest both general polyQ toxicity and mutant protein-specific effects. In this study, we report that the fat tumour suppressor gene mediates neurodegeneration induced by the polyQ protein Atrophin. We have monitored early transcriptional alterations in a Drosophila model of Dentatorubral-pallidoluysian Atrophy and found that polyQ Atrophins downregulate fat. Fat protects from neurodegeneration and Atrophin toxicity through the Hippo kinase cascade. Fat/Hippo signalling does not provoke neurodegeneration by stimulating overgrowth; rather, it alters the autophagic flux in photoreceptor neurons, thereby affecting cell homeostasis. Our data thus provide a crucial insight into the specific mechanism of a polyQ disease and reveal an unexpected neuroprotective role of the Fat/Hippo pathway.
PubMed ID
PubMed Central ID
PMC3049215 (PMC) (EuropePMC)
Related Publication(s)
Note
Slimming down fat makes neuropathic hippo: The Fat/Hippo tumor suppressor pathway protects adult neurons through regulation of autophagy.
Calamita and Fanto, 2011, Autophagy 7(8): 907--909 [FBrf0214041]
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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    EMBO J.
    Title
    The EMBO Journal
    Publication Year
    1982-
    ISBN/ISSN
    0261-4189
    Data From Reference
    Alleles (25)
    Genes (166)
    Human Disease Models (1)
    Cell Lines (1)
    Insertions (1)
    Transgenic Constructs (15)