New In Vitro Models to Study Amyotrophic Lateral Sclerosis.

Brain Pathology
Monika Myszczynska, Laura Ferraiuolo

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a complex multifactorial disorder, characterized by motor neuron loss with involvement of several other cell types, including astrocytes, oligodendrocytes and microglia. Studies in vivo and in in vitro models have highlighted that the contribution of non-neuronal cells to the disease is a primary event and ALS pathogenesis is driven by both cell-autonomous and non-cell autonomous mechanisms. The advancements in genetics and in vitro modeling of the past 10 years have dramatically changed the way we investigate the pathogenic mechanisms involved in ALS. The identification of mutations in transactive response DNA-binding protein gene (TARDBP), fused in sarcoma (FUS) and, more recently, a GGGGCC-hexanucleotide repeat expansion in chromosome 9 open reading frame 72 (C9ORF72) and their link with familial ALS have provided new avenues of investigation and hypotheses on the pathophysiology of this devastating disease. In the same years, from 2007 to present, in vitro technologies to model neurological disorders have also undergone impressive developments. The advent of induced pluripotent stem cells (iPSCs) gave the field of ALS the opportunity to finally model in vitro not only familial, but als...Continue Reading

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Citations

Jan 19, 2016·Brain Pathology·Caterina BendottiAntonio Migheli
May 18, 2018·Nature Reviews. Neurology·Akshata Almad, Nicholas J Maragakis
Nov 17, 2020·Frontiers in Neuroscience·Giovanna MorelloSebastiano Cavallaro
Jan 16, 2021·Tissue Engineering. Part C, Methods·Renata Vieira de SáR Jeroen Pasterkamp
Apr 4, 2021·International Journal of Molecular Sciences·Elisabeth TraiffortAmina Zahaf
Feb 21, 2019·ACS Chemical Neuroscience·Valle PalomoAna Martinez

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