Neuroprotective Effect of a DJ-1 Based Peptide in a Toxin Induced Mouse Model of Multiple System Atrophy

PloS One
Micaela Johanna GlatDaniel Offen

Abstract

Multiple System Atrophy (MSA) is a sporadic neurodegenerative disorder characterized by parkinsonism, cerebellar ataxia and dysautonomia, in various combinations. In MSA with parkinsonism (MSA-P), the degeneration is mainly restricted to the substantia nigra pars compacta and putamen. Studies have identified alterations in DJ-1 (PARK7), a key component of the anti-oxidative stress response, in Parkinson's disease (PD) and MSA patients. Previously we have shown that a short DJ-1-based peptide named ND-13, protected cultured cells against neurotoxic insults and improved behavioral outcome in animal models of Parkinson's disease (PD). In this study, we used the 3-Nitropropionic acid (3-NP)-induced mouse model of MSA and treated the animals with ND-13 in order to evaluate its therapeutic effects. Our results show that ND-13 protects cultured cells against oxidative stress generated by the mitochondrial inhibitor, 3-NP. Moreover, we show that ND-13 attenuates nigrostriatal degeneration and improves performance in motor-related behavioral tasks in 3-NP-treated mice. Our findings suggest a rationale for using ND-13 as a promising therapeutic approach for treatment of MSA.

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Citations

Jul 6, 2018·PloS One·Helio da SilvaLionel F Gamarra
Feb 18, 2020·Journal of Neural Transmission·Micaela Johanna GlatDaniel Offen
Sep 6, 2019·Journal of Clinical Medicine·Mariaelena Repici, Flaviano Giorgini
Dec 10, 2019·Biological Chemistry·Bradford G HillVictor M Darley-Usmar
Jan 5, 2021·Frontiers in Cell and Developmental Biology·Tingting WangYong Zhao
May 21, 2021·PLoS Biology·Koutarou NakamuraTakashi Shichita
Jun 27, 2021·Progress in Neurobiology·Maoxin Huang, Shengdi Chen

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Methods Mentioned

BETA
transgenic
fluorescence-activated cell sorter
PCR

Software Mentioned

Image
GraphPad Prism

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