Article
Disruption of mitochondrial membrane integrity induced by amyloid aggregates arising from variants of SOD1.
International journal of biological macromolecules - 1 Oct 2013
Oladzad Abbasabadi Azarakhsh, Javanian Asyeh, Nikkhah Maryam, Meratan Ali Akbar, Ghiasi Parisa, Nemat-Gorgani Mohsen
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder selectively affecting motor neurons; 90% of the total cases are sporadic, but 2% are associated with mutations in the gene coding for copper-zinc superoxide dismutase (SOD1). The causes of motor neuron death in ALS are poorly understood in general, but for SOD1-linked familial ALS (fALS), aberrant oligomerization of SOD1 mutant...
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