Article
Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.
Human molecular genetics - 15 Nov 2010
Ferri Alberto, Fiorenzo Paolo, Nencini Monica, Cozzolino Mauro, Pesaresi Maria Grazia, Valle Cristiana, Sepe Sara, Moreno Sandra, Carrì Maria Teresa
Abstract excerpt
Vulnerability of motoneurons in amyotrophic lateral sclerosis (ALS) arises from a combination of several mechanisms, including protein misfolding and aggregation, mitochondrial dysfunction and oxidative damage. Protein aggregates are found in motoneurons in models for ALS linked to a mutation in the gene coding for Cu,Zn superoxide dismutase (SOD1) and in ALS patients as well. Aggregation of mutant SOD1 in the...
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