A Database of Drosophila Genes & Genomes

FB2013_03, released May 7th, 2013
 

Reference Report

Reference
Citation Jung, J., Xu, K., Lessing, D., Bonini, N.M. (2009). Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3.  Hum. Mol. Genet. 18(24): 4843--4852. (Export to RIS)
FlyBase ID FBrf0209290
Publication Type Research paper
PubMed ID 19783548
PubMed Abstract Protein cleavage is a common feature in human neurodegenerative disease. Ataxin-3 protein with an expanded polyglutamine (polyQ) repeat causes spinocerebellar ataxia type-3 (SCA3), also called Machado-Joseph disease, and is cleaved in mammalian cells, transgenic mice and SCA3 patient brain tissue. However, the pathological significance of Ataxin-3 cleavage has not been carefully examined. To gain insight into the significance of Ataxin-3 cleavage, we developed a Drosophila SL2 cell-based model as well as transgenic fly models. Our data indicate that Ataxin-3 protein cleavage is conserved in the fly and may be caspase-dependent as reported previously. Importantly, comparison of flies expressing either wild-type or caspase-site mutant proteins indicates that Ataxin-3 cleavage enhances neuronal loss in vivo. This genetic in vivo confirmation of the pathological role of Ataxin-3 cleavage indicates that therapies targeting Ataxin-3 cleavage might slow disease progression in SCA3 patients.
DOI 10.1093/hmg/ddp456
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Language of Publication English
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Publication Type Journal
Abbreviation Hum. Mol. Genet.
Title Human Molecular Genetics
Publication Year 1992-
ISBN/ISSN 0964-6906
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