Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic. (October 2015)

• Record Type: Journal Article
• Authors: Mancuso, Renzo; Navarro, Xavier
• Is Part Of: Progress in neurobiology. Volume 133(2015:Oct.)
• Page Start: 1
• Page End: 26
• Publication Date: 2015-10
• Subjects: AAV adeno-associated virus -- ALS amyotrophic lateral sclerosis -- AMPA α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid -- ChAT choline acetyltransferase -- CHOP C/EBP homologous protein -- CMAP compound muscle action potential -- CNTF ciliary neurotrophic factor -- EAAT excitatory amino-acid transporter -- EMG electromyography -- ER endoplasmic reticulum -- fALS familiar ALS -- FTLD frontotemporal lobal degeneration -- FUS fused in sarcoma -- GDNF glial derived neurotrophic factor -- IGF-1 insulin-like growth factor 1 -- iPS induced pluripotent stem -- KAP3 kinesin-associated protein 3 -- KCC2 potassium-chloride co-transporter 2 -- MN motoneuron -- MND motoneuron diseases -- MRI magnetic resonance image -- MSC mesenchymal stem cell -- MUNE motor unit number estimation -- NMDA N-methyl-d-aspartate -- NSC neural stem cell -- ROS reactive oxygen species -- sALS sporadic ALS -- SMA spinal muscular atrophy -- SOD1 superoxide dismutase 1 -- TDP-43 TAR-DNA binding protein 43 -- TMS transcranial magnetic stimulation -- UPR unfolded protein response; Amyotrophic lateral sclerosis -- Motoneurons disease -- Neurodegeneration -- Neuromuscular junction -- Neuroprotection; Neurobiology -- Periodicals; Neurology -- Periodicals; Neurology -- Periodicals; Neurobiologie -- Périodiques; 612.8
• DOI: 10.1016/j.pneurobio.2015.07.004
• Languages: English
• ISSNs: 0301-0082

Abstract

Graphical abstract: Highlights: ALS is a neurodegenerative disease affecting upper and lower motoneurons. Experimental models are instrumental in leading to translational research. The review covers the diverse aspects of ALS physiopathology. The most relevant and novel therapeutic strategies are summarized. Abstract: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of upper and lower motoneurons, leading to muscle weakness and paralysis, and finally death. Considerable recent advances have been made in basic research and preclinical therapeutic attempts using experimental models, leading to increasing clinical and translational research in the context of this disease. In this review we aim to summarize the most relevant findings from a variety of aspects about ALS, including evaluation methods, animal models, pathophysiology, and clinical findings, with particular emphasis in understanding the role of every contributing mechanism to the disease for elucidating the causes underlying degeneration of motoneurons and the development of new therapeutic strategies.