TY - JOUR
T1 - Autophagy in motor neuron disease
T2 - Key pathogenetic mechanisms and therapeutic targets
AU - Cipolat Mis, Maria Sara
AU - Brajkovic, Simona
AU - Frattini, Emanuele
AU - Di Fonzo, Alessio
AU - Corti, Stefania
PY - 2016/4/1
Y1 - 2016/4/1
N2 - Autophagy is a lysosome-dependant intracellular degradation process that eliminates long-lived proteins as well as damaged organelles from the cytoplasm. An increasing body of evidence suggests that dysregulation of this system plays a pivotal role in the etiology and/or progression of neurodegenerative diseases including motor neuron disorders. Herein, we review the latest findings that highlight the involvement of autophagy in the pathogenesis of amyotrophic lateral sclerosis (ALS) and the potential role of this pathway as a target of therapeutic purposes. Autophagy promotes the removal of toxic, cytoplasmic aggregate-prone pathogenetic proteins, enhances cell survival, and modulates inflammation. The existence of several drugs targeting this pathway can facilitate the translation of basic research to clinical trials for ALS and other motor neuron diseases.
AB - Autophagy is a lysosome-dependant intracellular degradation process that eliminates long-lived proteins as well as damaged organelles from the cytoplasm. An increasing body of evidence suggests that dysregulation of this system plays a pivotal role in the etiology and/or progression of neurodegenerative diseases including motor neuron disorders. Herein, we review the latest findings that highlight the involvement of autophagy in the pathogenesis of amyotrophic lateral sclerosis (ALS) and the potential role of this pathway as a target of therapeutic purposes. Autophagy promotes the removal of toxic, cytoplasmic aggregate-prone pathogenetic proteins, enhances cell survival, and modulates inflammation. The existence of several drugs targeting this pathway can facilitate the translation of basic research to clinical trials for ALS and other motor neuron diseases.
KW - Amyotrophic lateral sclerosis
KW - Authophagy
KW - Protein aggregation
UR - http://www.scopus.com/inward/record.url?scp=84957045630&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84957045630&partnerID=8YFLogxK
U2 - 10.1016/j.mcn.2016.01.012
DO - 10.1016/j.mcn.2016.01.012
M3 - Article
AN - SCOPUS:84957045630
VL - 72
SP - 84
EP - 90
JO - Molecular and Cellular Neurosciences
JF - Molecular and Cellular Neurosciences
SN - 1044-7431
ER -