TY - JOUR
T1 - Amyotrophic lateral sclerosis
T2 - New insights into underlying molecular mechanisms and opportunities for therapeutic intervention
AU - Cozzolino, Mauro
AU - Pesaresi, Maria Grazia
AU - Gerbino, Valeria
AU - Grosskreutz, Julian
AU - Carrì, Maria Teresa
PY - 2012/11/1
Y1 - 2012/11/1
N2 - Recent years have witnessed a renewed interest in the pathogenic mechanisms of amyotrophic lateral sclerosis (ALS), a late-onset progressive degeneration of motor neurons. The discovery of new genes associated with the familial form of the disease, along with a deeper insight into pathways already described for this disease, has led scientists to reconsider previous postulates. While protein misfolding, mitochondrial dysfunction, oxidative damage, defective axonal transport, and excitotoxicity have not been dismissed, they need to be re-examined as contributors to the onset or progression of ALS in the light of the current knowledge that the mutations of proteins involved in RNA processing, apparently unrelated to the previous old partners, are causative of the same phenotype. Thus, newly envisaged models and tools may offer unforeseen clues on the etiology of this disease and hopefully provide the key to treatment.
AB - Recent years have witnessed a renewed interest in the pathogenic mechanisms of amyotrophic lateral sclerosis (ALS), a late-onset progressive degeneration of motor neurons. The discovery of new genes associated with the familial form of the disease, along with a deeper insight into pathways already described for this disease, has led scientists to reconsider previous postulates. While protein misfolding, mitochondrial dysfunction, oxidative damage, defective axonal transport, and excitotoxicity have not been dismissed, they need to be re-examined as contributors to the onset or progression of ALS in the light of the current knowledge that the mutations of proteins involved in RNA processing, apparently unrelated to the previous old partners, are causative of the same phenotype. Thus, newly envisaged models and tools may offer unforeseen clues on the etiology of this disease and hopefully provide the key to treatment.
UR - http://www.scopus.com/inward/record.url?scp=84865654196&partnerID=8YFLogxK
U2 - 10.1089/ars.2011.4328
DO - 10.1089/ars.2011.4328
M3 - Scientific review articles
C2 - 22413952
AN - SCOPUS:84865654196
SN - 1523-0864
VL - 17
SP - 1277
EP - 1330
JO - Antioxidants and Redox Signaling
JF - Antioxidants and Redox Signaling
IS - 9
ER -