CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations

Acta Neuropathologica

Volumn 102, Issue 4, 2001, Pages 293-305

  Jaarsma, Dick ^a   Rognoni, Francesca ^a   Van Duijn, Wim ^b   Verspaget, Hein W ^b   Haasdijk, Elize D ^a   Holstege, Jan C ^a  

^a ERASMUS UNIVERSITY ROTTERDAM   (Netherlands)

^b LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Amyotrophic lateral sclerosis; Degeneration; Mitochondria; Motor neuron; Superoxide dismutase

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; CYTOCHROME C; SUPEROXIDE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL VACUOLE; CONFOCAL MICROSCOPY; CONTROLLED STUDY; ELECTRON MICROSCOPY; ENZYME LOCALIZATION; GENE EXPRESSION; GENE MUTATION; GENE TRANSLOCATION; MITOCHONDRIAL MEMBRANE; MITOCHONDRION; MOUSE; NERVE DEGENERATION; NONHUMAN; OUTER MEMBRANE; PRIORITY JOURNAL; TRANSGENIC MOUSE;

EID: 0034821922     PISSN: 00016322     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004010100399     Document Type: Article

References (39)

1. The effect of oncotic pressure on heart muscle mitochondria
2. Enhanced superoxide dismutase-2 immunoreactivity of astrocytes and occasional neurons in amyotrophic lateral sclerosis
3. Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death
4. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis
5. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
6. Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells
7. Superoxide dismutase catalyzes nitration of tyrosines by peroxynitrite in the rod and head domains of neurofilament-L
8. 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)
9. 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
10. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase
11. Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
12. Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase
13. Superoxide dismutases
14. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation
15. The effects of colloids on the appearance and substrate permeability of rat liver mitochondria
16. Glutamate receptor subunits at mossy fiber-unipolar brush cell synapses: Light and electron microscopic immunocytochemical study in the cerebellar cortex of rat and cat
17. Induction of c-Jun immunoreactivity in spinal cord and brainstem neurons in a transgenic mouse model for amyotrophic lateral sclerosis
18. Degeneration of spinal interneurons in addition to motoneurons in a trangenic mouse model for ALS
19. The antioxidant N-acetylcysteine does not delay disease onset and death in a transgenic mouse model of amyotrophic lateral sclerosis
20. Cu/Zn superoxide dismutase (SOD1) over-expression in mice causes mitochondrial degeneration, axonal degeneration and premature motoneuron death, and accelerates the development of motoneuron disease in mice expressing fALS-mutant SOD1
21. Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
22. Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis
23. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1
24. Mutations in SOD1 associated with amyotrophic lateral sclerosis cause novel protein interactions
25. The 'ins' and 'outs' of mitochondrial membrane channels
26. Normal binding and reactivity of copper in mutant superoxide dismutase isolated from amyotrophic lateral sclerosis patients
27. Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds
28. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
29. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis
30. Mutations in all five exons of SOD-1 may cause ALS
31. Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement
32. ALS: Molecular clues to the jigsaw puzzle of neuronal degeneration
33. Intracellular localization of the copper-zinc and manganese superoxide dismutases in rat liver parenchymal cells
34. Freeze-substitution and Lowicryl HM20 embedding of fixed rat brain: Suitability for immunogold ultrastructural localization of neural antigens
35. Outer mitochondrial membrane permeability can regulate coupled respiration and cell survival
36. Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis
37. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
38. Brain cytochrome oxidase: Functional significance and bigenomic regulation in the CNS
39. A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in K-m for hydrogen peroxide