Inhibition of the deubiquitinase USP8 corrects a Drosophila PINK1 model of mitochondria dysfunction.
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ABSTRACT:
SUBMITTER: von Stockum S
PROVIDER: S-EPMC6467245 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
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Inhibition of the deubiquitinase USP8 corrects a Drosophila PINK1 model of mitochondria dysfunction.
von Stockum Sophia S Sanchez-Martinez Alvaro A Corrà Samantha S Chakraborty Joy J Marchesan Elena E Locatello Lisa L Da Rè Caterina C Cusumano Paola P Caicci Federico F Ferrari Vanni V Costa Rodolfo R Bubacco Luigi L Rasotto Maria Berica MB Szabo Ildiko I Whitworth Alexander J AJ Scorrano Luca L Ziviani Elena E
Life science alliance 20190415 2
Aberrant mitochondrial dynamics disrupts mitochondrial function and contributes to disease conditions. A targeted RNA interference screen for deubiquitinating enzymes (DUBs) affecting protein levels of multifunctional mitochondrial fusion protein Mitofusin (MFN) identified USP8 prominently influencing MFN levels. Genetic and pharmacological inhibition of USP8 normalized the elevated MFN protein levels observed in PINK1 and Parkin-deficient models. This correlated with improved mitochondrial func ...[more]
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