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ORIGINAL ARTICLES
Published online: 2018-08-09
Submitted: 2018-06-18
Accepted: 2018-07-10
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Evaluation of the role of an antioxidant gene in NSC-34 motor neuron-like cells as a model of a motor neuron disease

Aziza Rashed Alrafiah
DOI: 10.5603/FM.a2018.0072
·
Pubmed: 30106460

open access

Ahead of Print
ORIGINAL ARTICLES
Published online: 2018-08-09
Submitted: 2018-06-18
Accepted: 2018-07-10

Abstract

Background: Spinal muscular atrophy is a rare genetic disease, which primarily affects motor neurons and predominantly occurs in children. To date, alternatives for the treatment of the disease have been controversial. Spinal muscular atrophy has a multi-factorial etiology, with mitochondrial oxidative stress considered as the crucial pathogenic mechanism. To determine the mechanisms underlying the loss of motor neurons, NSC-34 motor neuron-like cells are often used as an in vitro model of spinal muscular atrophy. As Plastin 3 (PLS3) has been demonstrated as a modifier of spinal muscular atrophy, the aim of the current study was to evaluate the neuroprotective effect of plastin 3 in NSC-34 cells. Materials and methods: Plastin 3 was overexpressed in human embryonic kidney 293T cells and NSC-34 cells via lentiviral transduction. NSC-34 cells transduced with a lentiviral vector carrying the gene for LacZ β-galactosidase served as a control. Oxidative stress was then induced by depriving cells of serum, and the protective effect of plastin 3 was assessed using a cellular reactive oxygen species detection assay. Results: While plastin 3 was successfully overexpressed in human embryonic kidney 293T cells and NSC-34 cells, upregulation of this protein did not significantly decrease oxidative stress in serum-deprived NSC-34 cells relative to controls. Conclusions: Plastin 3 overexpression in NSC-34 cells did not elicit an antioxidative effect following serum deprivation.

Abstract

Background: Spinal muscular atrophy is a rare genetic disease, which primarily affects motor neurons and predominantly occurs in children. To date, alternatives for the treatment of the disease have been controversial. Spinal muscular atrophy has a multi-factorial etiology, with mitochondrial oxidative stress considered as the crucial pathogenic mechanism. To determine the mechanisms underlying the loss of motor neurons, NSC-34 motor neuron-like cells are often used as an in vitro model of spinal muscular atrophy. As Plastin 3 (PLS3) has been demonstrated as a modifier of spinal muscular atrophy, the aim of the current study was to evaluate the neuroprotective effect of plastin 3 in NSC-34 cells. Materials and methods: Plastin 3 was overexpressed in human embryonic kidney 293T cells and NSC-34 cells via lentiviral transduction. NSC-34 cells transduced with a lentiviral vector carrying the gene for LacZ β-galactosidase served as a control. Oxidative stress was then induced by depriving cells of serum, and the protective effect of plastin 3 was assessed using a cellular reactive oxygen species detection assay. Results: While plastin 3 was successfully overexpressed in human embryonic kidney 293T cells and NSC-34 cells, upregulation of this protein did not significantly decrease oxidative stress in serum-deprived NSC-34 cells relative to controls. Conclusions: Plastin 3 overexpression in NSC-34 cells did not elicit an antioxidative effect following serum deprivation.

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Keywords

oxidative stress, plastin 3, NSC-34 cells, lentiviral vectors, spinal muscular atrophy

About this article
Title

Evaluation of the role of an antioxidant gene in NSC-34 motor neuron-like cells as a model of a motor neuron disease

Journal

Folia Morphologica

Issue

Ahead of Print

Published online

2018-08-09

DOI

10.5603/FM.a2018.0072

Pubmed

30106460

Keywords

oxidative stress
plastin 3
NSC-34 cells
lentiviral vectors
spinal muscular atrophy

Authors

Aziza Rashed Alrafiah

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