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Citation
Chen, Y., Yang, M., Deng, J., Chen, X., Ye, Y., Zhu, L., Liu, J., Ye, H., Shen, Y., Li, Y., Rao, E.J., Fushimi, K., Zhou, X., Bigio, E.H., Mesulam, M., Xu, Q., Wu, J.Y. (2011). Expression of human FUS protein in Drosophila leads to progressive neurodegeneration.  Protein Cell 2(6): 477--486.
FlyBase ID
FBrf0214276
Publication Type
Research paper
Abstract

Mutations in the Fused in sarcoma/Translated in liposarcoma gene (FUS/TLS, FUS) have been identified among patients with amyotrophic lateral sclerosis (ALS). FUS protein aggregation is a major pathological hallmark of FUS proteinopathy, a group of neurodegenerative diseases characterized by FUS-immunoreactive inclusion bodies. We prepared transgenic Drosophila expressing either the wild type (Wt) or ALS-mutant human FUS protein (hFUS) using the UAS-Gal4 system. When expressing Wt, R524S or P525L mutant FUS in photoreceptors, mushroom bodies (MBs) or motor neurons (MNs), transgenic flies show age-dependent progressive neural damages, including axonal loss in MB neurons, morphological changes and functional impairment in MNs. The transgenic flies expressing the hFUS gene recapitulate key features of FUS proteinopathy, representing the first stable animal model for this group of devastating diseases.

PubMed ID
PubMed Central ID
PMC3563268 (PMC) (EuropePMC)
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    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Protein Cell
    Title
    Protein & Cell
    ISBN/ISSN
    1674-800X 1674-8018
    Data From Reference
    Alleles (8)
    Genes (4)
    Human Disease Models (1)
    Natural transposons (1)
    Insertions (2)
    Experimental Tools (3)
    Transgenic Constructs (6)