Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation

Neuropathology

Volumn 21, Issue 1, 2001, Pages 82-92

  Shibata, Noriyuki ^a  

^a TOKYO WOMEN'S MEDICAL UNIVERSITY   (Japan)

Author keywords

Amyotrophic lateral sclerosis; Carbonyls; Motor neuron degeneration; Oxidative stress; Superoxide dismutase; Transgenic mice

Indexed keywords

GLUTAMIC ACID; PEROXYNITRITE; SUPEROXIDE DISMUTASE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; APOPTOSIS; ARTICLE; BRAIN MITOCHONDRION; CALCIUM HOMEOSTASIS; CYTOSKELETON; DISEASE MODEL; ENDOPLASMIC RETICULUM; FAMILIAL DISEASE; GENE MUTATION; GENE OVEREXPRESSION; HUMAN; MOTONEURON; MOUSE; NERVE CELL DEGENERATION; NEUROTOXICITY; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN AGGREGATION; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; DISEASE MODELS, ANIMAL; MICE; MICE, TRANSGENIC; SUPEROXIDE DISMUTASE;

EID: 0035070937     PISSN: 09196544     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1440-1789.2001.00361.x     Document Type: Article

References (51)

1. Superoxide dismutase-1 mutation-related neurotoxicity in familial amyotrophic lateral sclerosis
2. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
3. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
4. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis
5. SOD1 immunoreactive precipitates in mice transgenic for human SOD1 with and without mutations
6. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
7. Inhibition of ICE slows ALS in mice
8. Generation of mutant SOD1-expressing mice
9. Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild-type human SOD: A model of familial amyotrophic lateral sclerosis (FALS)
10. A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis
11. The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease
12. Familial amyotrophic lateral sclerosis with widespread vacuolation and hyaline inclusions
13. Presence of Cu/Zn superoxide dismutase (SOD1) immunoreactivity in neuronal hyaline inclusions in spinal cords from mice carrying a transgene for Gly93Ala mutant human Cu/Zn SOD
14. Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement
15. Cytopathology of the motor neuron
16. Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis
17. Morphological aspects of superoxide dismutase-1 mutation in amyotrophic lateral sclerosis and its transgenic mouse model
18. Elevated 'hydroxyl radical' generation in vivo in an animal model of amyotrophic lateral sclerosis
19. Enchanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis
20. Benefit of vitamin E, riluzole, and gabapentine in a transgenic model of familial amyotrophic lateral sclerosis
21. Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
22. Therapeutic benefits of putrescine-modified catalase in a transgenic mouse model of familial amyotrophic lateral sclerosis
23. 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
24. The antioxidant N-acetylcysteine does not delay disease onset and death in a transgenic mouse model of amyotrophic lateral sclerosis
25. Nonoxidative protein glycation is implicated in familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation
26. Peroxynitrite-mediated oxidative protein modification
27. Increased 3-nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutation
28. Nitration of the low molecular weight neurofilament is equivalent in sporadic amyotrophic lateral sclerosis and control cervical spinal cord
29. Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
30. 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
31. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis
32. Knockout of glutamate transporters reveals a major role for astroglial transport in excitotoxicity and clearance of glutamate
33. Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis
34. Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice
35. Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
36. Parvalbumin and calbindin D-28k in the human motor neuron disease
37. Familial amyotrophic lateral sclerosis with or without mutation in the Cu/Zn superoxide dismutase gene
38. Familial amyotrophic lateral sclerosis with the L126S mutation in the copper/zinc superoxide dismutase (SOD1) gene
39. Molecular pathological studies of familial amyotrophic lateral sclerosis
40. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
41. Lack of apoptosis in mice with ALS
42. Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
43. Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis
44. Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis
45. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis
46. Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis
47. Immunohistochemical study of protein-bound carbonyls in the spinal cord of transgenic mice expressing G93A mutant superoxide dismutase-1 (SOD1)
48. Protein modification by the lipid peroxidation product 4-hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients
49. Inhibitory effect of nonenzymatic glycosylation on ubiquitination and ubiquitin-mediated degradation of lysozyme
50. Bcl-2: Prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis