Amyotrophic lateral sclerosis free radicals

Calabrese V. 2007. Highlight commentary on Redox proteomics analysis of oxidatively 3 modified proteins in G93A-SOD1 transgenic mice-A model of 4 familial amyotrophic lateral sclerosis. Free Radical Biol Med 43 160-162. 

Perluigi M., Poon H. F., Hensley K., Pierce W. M., Klein J. B., Calabrese V., De Marco C., and Butterfield D. A. (2005). Proteomic analysis of 4-hydroxy-2-nonenal-modified proteins in G93A-SOD1 transgenic mice - A model of familial amyotrophic lateral sclerosis. Free Radical Biol. Med. 38 960-968. 

Bruijn, L. I., Beal, M. F., Becher, M. W. et al. Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant. Proc. Natl Acad. Sci. U.S.A. 94 7606-7611,1997. 

Frontotemporal dementia involves an early and primary degenerative process of frontal and/or temporal cortex. Several disorders fall under this rubric, such as Pick s disease and the dementia associated with amyotrophic lateral sclerosis (ALS). ALS is a degenerative disease of upper motor neurons that is sometimes accompanied by a frontal lobe dementia (Vercelletto et al. 1999 Abe et al. 1997). ALS has been associated with mutations in the free radical scavenging enzyme superoxide dismutase 1 (Price et al. 1997). Pick s disease is associated histologically with a loss of neurons and cytoplasmic Pick bodies in surviving neurons. 

The excessive activity of excitatory amino acids, such as L-glutamate and L-aspartate, followed by elevation of intracellular free Ca2+ concentration and accumulation of free radicals has been postulated to underlie the neurodegeneration that occurs after ischemic insults and trauma. Additionally, an excitotoxic component has been shown to play an important role in the pathogenesis of chronic neurodegenerative disorders, such as Alzheimer s disease, Parkinson s disease, amyotrophic lateral sclerosis, and Huntington s disease, which are characterized by progressive loss of neuronal elements. 

Yim MB, Karrg J-H, Kwak H-S, Chock PB, Stadtman ER (1996) A gairr-of-furrcdorr of arr amyotrophic lateral sclerosis-associated Cu, Zrr superoxide dismutase mutant Arr errhancement of free radical fornradorr due to a decreased hr BHn for hydrogen peroxide. Proc Natl Acad Sci USA 93 5709-5714. 

Liu R, Li B, Flanagan SW, Oberley LW, Gozal D, Oiu M (2002) Increased mitochondrial antiox-idative activity or decreased oxygen free radical propagation prevent mutant SODl-mediated motor neuron cell death and increase amyotrophic lateral sclerosis-like transgenic mouse... 

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