open access

Vol 78, No 1 (2019)
Original article
Submitted: 2018-06-18
Accepted: 2018-07-10
Published online: 2018-08-09
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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

A. R. Alrafiah12
·
Pubmed: 30106460
·
Folia Morphol 2019;78(1):1-9.
Affiliations
  1. Department of Medical Laboratory Technology, Faculty of Applied Medical Sciences, Jeddah, Saudi Arabia
  2. Neuroscience Research Unit, King Abdulaziz University, Faculty of Medicine, Branch of Sulaymaniyah, Jeddah, Saudi Arabia

open access

Vol 78, No 1 (2019)
ORIGINAL ARTICLES
Submitted: 2018-06-18
Accepted: 2018-07-10
Published online: 2018-08-09

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 aetiology, 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 PLS3 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 PLS3 was assessed using a cellular reactive oxygen species detection assay. 

Results: While PLS3 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 aetiology, 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 PLS3 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 PLS3 was assessed using a cellular reactive oxygen species detection assay. 

Results: While PLS3 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

Vol 78, No 1 (2019)

Article type

Original article

Pages

1-9

Published online

2018-08-09

Page views

2185

Article views/downloads

1450

DOI

10.5603/FM.a2018.0072

Pubmed

30106460

Bibliographic record

Folia Morphol 2019;78(1):1-9.

Keywords

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

Authors

A. R. Alrafiah

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