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Parkin expression reverses mitochondrial dysfunction in fused in sarcoma-induced amyotrophic lateral sclerosis

Authors
Cha, S. J.Choi, H. -J.Kim, H. -J.Choi, E. J.Song, K. -H.Im, D. S.Kim, K.
Issue Date
Feb-2020
Publisher
Blackwell Publishing Inc.
Keywords
amyotrophic lateral sclerosis; FUS; parkin; mitochondrial dysfunction; Drosophila
Citation
Insect Molecular Biology, v.29, no.1, pp 56 - 65
Pages
10
Journal Title
Insect Molecular Biology
Volume
29
Number
1
Start Page
56
End Page
65
URI
https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/3165
DOI
10.1111/imb.12608
ISSN
0962-1075
1365-2583
Abstract
Fused in sarcoma (FUS) is a DNA/RNA-binding protein associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. The exact molecular mechanisms by which FUS results in neurotoxicity have not yet been fully elucidated. Here, we found that parkin is a genetic suppressor of defective phenotypes induced by exogenous human wild type FUS in Drosophila. Although parkin overexpression did not modulate the FUS protein expression level, the locomotive defects in FUS-expressing larvae and adult flies were rescued by parkin expression. We found that FUS expression in muscle tissues resulted in a reduction of the levels and assembly of mitochondrial complex I and III subunits, as well as decreased ATP. Remarkably, expression of parkin suppressed these mitochondrial dysfunctions. Our results indicate parkin as a neuroprotective regulator of FUS-induced proteinopathy by recovering the protein levels of mitochondrial complexes I and III. Our findings on parkin-mediated neuroprotection may expand our understanding of FUS-induced ALS pathogenesis.
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College of Medical Sciences (Department of Pharmaceutical Engineering)
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