Research On Overexpression Of PINK1 Improve Mitochondrial Function In SCA3/MJD Transgenic Drosophila Model

Objective: Spinocerebellar ataxia type 3(SCA3), also known as Machado Joseph disease(MJD), caused by larger expanded CAG repeat sizes in the MJD1 gene, is an autosomal dominant neurodegenerative disorder of late onset.Overexpression of PINK1 can prevent the occurrence of Parkinson’s disease by protecting against mitochondrial oxidative stress, while the role of overexpression of PINK1 is unknown in SCA3 / MJD. The purpose of this paper is to confer the protective role of PINK1 overexpression in SCA3/MJD transgenic Drosophila model.Methods: SCA3/MJD flies were constructed by using Mhc-Gal4 to drive the expression of MJDtr-Q78. Here, we describe the phenotype, the mitochondrial morphology, ATP and Real-Time PCR caused by overexpression of PINK1 and RNA interference(RNAi) of PINK1 are expressed in SCA3/MJD flies respectively.Results: Our results indicate that SCA3/MJD flies show defective wing positions,swollen mitochondria, and ATP deficits compared with control flies.These phenotypes could be rescued by the PINK1 overexpression but not the PINK1 RNAi.Conclusion: Our results suggest that overexpression of PINK1 notably protects SCA3/MJD transgenic Drosophila models, whereas PINK1 RNAi significantly aggravates SCA3/MJD transgenic Drosophila models. And this protection role of PINK1 in SCA3/MJD transgenic Drosophila model may be associated with the improvement of mitochondrial function.