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

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.

journal_name

Folia Morphol (Warsz)

journal_title

Folia morphologica

authors

Alrafiah AR

doi

10.5603/FM.a2018.0072

subject

Has Abstract

pub_date

2019-01-01 00:00:00

pages

1-9

issue

1

eissn

0015-5659

issn

1644-3284

pii

VM/OJS/J/58597

journal_volume

78

pub_type

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