Mice Overexpressing Both Non-Mutated Human SOD1 and Mutated SOD1(G93A) Genes: A Competent Experimental Model for Studying Iron Metabolism in Amyotrophic Lateral Sclerosis

Frontiers in Molecular Neuroscience
Anna GajowiakPaweł Lipiński

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by degeneration and loss of motor neurons in the spinal cord, brainstem and motor cortex. Up to 10% of ALS cases are inherited (familial, fALS) and associated with mutations, frequently in the superoxide dismutase 1 (SOD1) gene. Rodent transgenic models of ALS are often used to elucidate a complex pathogenesis of this disease. Of importance, both ALS patients and animals carrying mutated human SOD1 gene show symptoms of oxidative stress and iron metabolism misregulation. The aim of our study was to characterize changes in iron metabolism in one of the most commonly used models of ALS - transgenic mice overexpressing human mutated SOD1(G93A) gene. We analyzed the expression of iron-related genes in asymptomatic, 2-month-old and symptomatic, 4-month-old SOD1(G93A) mice. In parallel, respective age-matched mice overexpressing human non-mutated SOD1 transgene and control mice were analyzed. We demonstrate that the overexpression of both SOD1 and SOD1(G93A) genes account for a substantial increase in SOD1 protein levels and activity in selected tissues and that not all the changes in iron metabolism genes expression are specific for the over...Continue Reading

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Citations

Feb 9, 2018·Journal of Translational Medicine·Driton Vela
Jan 9, 2020·Journal of Neural Transmission·David DevosUNKNOWN FAIRPARK-II and FAIRALS-II studygroups
Apr 6, 2019·Frontiers in Neuroscience·Malgorzata Halon-GolabekJedrzej Antosiewicz
Sep 21, 2018·Pharmaceuticals·Veronica FioritoEmanuela Tolosano
Feb 7, 2019·Frontiers in Neuroscience·Karina JoppePaul Lingor

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Methods Mentioned

BETA
transgenic
electrophoresis
light microscopy
PCR
protein assay

Software Mentioned

Statistica
Quantity One

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