Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with amyotrophic lateral sclerosis (ALS): immunohistochemical studies on sporadic ALS and familial ALS with superoxide dismutase 1 gene mutation

2003 ◽  
Vol 106 (2) ◽  
pp. 112-120 ◽  
Author(s):  
Shinsuke Kato ◽  
Hiroshi Funakoshi ◽  
Toshikazu Nakamura ◽  
Masako Kato ◽  
Imaharu Nakano ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Growth Factor ◽  
Superoxide Dismutase 1 ◽  
Sporadic Als ◽  
Familial Als ◽  
Immunohistochemical Studies ◽  
Hepatocyte Growth ◽  
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
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