New Insights on the Mechanisms of Disease Course Variability in ALS from Mutant SOD1 Mouse Models.

Brain Pathology
Giovanni NardoCaterina Bendotti

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous disease in terms of progression rate and survival. This is probably one of the reasons for the failure of many clinical trials and the lack of effective therapies. Similar variability is also seen in SOD1(G93A) mouse models based on their genetic background. For example, when the SOD1(G93A) transgene is expressed in C57BL6 background the phenotype is mild with slower disease progression than in the 129Sv mice expressing the same amount of transgene but showing faster progression and shorter lifespan. This review summarizes and discusses data obtained from the analysis of these two mouse models under different aspects such as the motor phenotype, neuropathological alterations in the central nervous system (CNS) and peripheral nervous system (PNS) and the motor neuron autonomous and non-cell autonomous mechanisms with the aim of finding elements to explain the different rates of disease progression. We also discuss the identification of promising prognostic biomarkers by comparative analysis of the two ALS mouse models. This analysis might possibly suggest new strategies for effective therapeutic intervention in ALS to slow significantly or even block the course of the disease.

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Citations

Jan 19, 2016·Brain Pathology·Caterina BendottiAntonio Migheli
Apr 22, 2017·Frontiers in Molecular Neuroscience·Melania FilaretiValentina Bonetto
Nov 7, 2018·Journal of Neurogenetics·Sean J MillerJeffrey D Rothstein
Mar 3, 2018·Journal of Neuroinflammation·Antonio VallarolaCaterina Bendotti
May 14, 2020·Cells·Marco CeccantiMaurizio Inghilleri
Sep 12, 2018·Chinese Medical Journal·Xiao-Sun SunXu-Sheng Huang
Jul 24, 2018·Evidence-based Complementary and Alternative Medicine : ECAM·Sunjung Park, Eun Jin Yang
Oct 17, 2018·Neural Regeneration Research·Jessica R MorriceChristopher A Shaw
Jul 27, 2018·Mediators of Inflammation·MuDan Cai, Eun Jin Yang
Aug 28, 2020·Frontiers in Cellular Neuroscience·James N SleighGiampietro Schiavo
Oct 9, 2016·Journal of Neuroinflammation·Giovanni NardoCaterina Bendotti
Aug 11, 2018·Molecular Neurodegeneration·Giovanni NardoCaterina Bendotti
Nov 9, 2018·Molecular Neurodegeneration·Irene ZubiriAndrea Malaspina
Jan 28, 2021·International Journal of Molecular Sciences·Hilal CihankayaVeronika Matschke
Jan 3, 2020·ACS Chemical Neuroscience·Katelyn M BaumerBryan F Shaw
Sep 3, 2021·Neural Regeneration Research·Bridget Martinez, Philip V Peplow
Dec 23, 2021·Cellular and Molecular Life Sciences : CMLS·Paola FabbrizioGiovanni Nardo

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