scholarly journals 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

1997 ◽  
Vol 94 (14) ◽  
pp. 7606-7611 ◽  
Author(s):  
L. I. Bruijn ◽  
M. F. Beal ◽  
M. W. Becher ◽  
J. B. Schulz ◽  
P. C. Wong ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Hydroxyl Radicals ◽  
Tyrosine Nitration ◽  
Superoxide Dismutase 1 ◽  
Familial Als ◽  
Lateral Sclerosis

scholarly journals Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant

1998 ◽  
Vol 95 (16) ◽  
pp. 9631-9636 ◽  
Author(s):  
Toni L. Williamson ◽  
Lucie I. Bruijn ◽  
Qinzhang Zhu ◽  
Karen L. Anderson ◽  
Scott D. Anderson ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Motor Neurons ◽  
Selective Vulnerability ◽  
Superoxide Dismutase 1 ◽  
Filament Assembly ◽  
Progression Of Disease ◽  
Familial Als ◽  
Sod1 Mutant ◽  
Lateral Sclerosis

Mutations in superoxide dismutase 1 (SOD1), the only proven cause of amyotrophic lateral sclerosis (ALS), provoke disease through an unidentified toxic property. Neurofilament aggregates are pathologic hallmarks of both sporadic and SOD1-mediated familial ALS. By deleting NF-L, the major neurofilament subunit required for filament assembly, onset and progression of disease caused by familial ALS-linked SOD1 mutant G85R are significantly slowed, while selectivity of mutant-mediated toxicity for motor neurons is reduced. In NF-L-deleted animals, levels of the two remaining neurofilament subunits, NF-M and NF-H, are markedly reduced in axons but are elevated in motor neuron cell bodies. Thus, while neither perikaryal nor axonal neurofilaments are essential for SOD1-mediated disease, the absence of assembled neurofilaments both diminishes selective vulnerability and slows SOD1G85R mutant-mediated toxicity to motor neurons.

scholarly journals Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis

2011 ◽  
Vol 121 (5) ◽  
pp. 623-634 ◽  
Author(s):  
Karin Forsberg ◽  
Peter M. Andersen ◽  
Stefan L. Marklund ◽  
Thomas Brännström
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Superoxide Dismutase 1 ◽  
Lateral Sclerosis

scholarly journals Phenotype of Transgenic Mice Carrying a Very Low Copy Number of the Mutant Human G93A Superoxide Dismutase-1 Gene Associated with Amyotrophic Lateral Sclerosis

PLoS ONE ◽  
2014 ◽  
Vol 9 (6) ◽  
pp. e99879 ◽  
Author(s):  
Jeffrey S. Deitch ◽  
Guillermo M. Alexander ◽  
Andrew Bensinger ◽  
Steven Yang ◽  
Juliann T. Jiang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Transgenic Mice ◽  
Copy Number ◽  
Superoxide Dismutase 1 ◽  
Low Copy Number ◽  
Lateral Sclerosis
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