Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment

Journal of Neuroscience

Volumn 21, Issue 10, 2001, Pages 3369-3374

  Pramatarova, Albéna ^a,b   Laganière, Janet ^a   Roussel, Julie ^a   Brisebois, Katéri ^a   Rouleau, Guy A ^a  

^a MONTREAL GENERAL HOSPITAL   (Canada)

^b NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

Author keywords

Amyotrophic lateral sclerosis; Cell death; Mutant SOD1; Neurofilament promoter; Neuronal expression; Superoxide dismutase; Transgenic mice

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; MUTANT PROTEIN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; EMBRYO; FAMILIAL DISEASE; GENE MUTATION; HUMAN; MOTONEURON; MOTOR DYSFUNCTION; MOUSE; NERVE CELL NECROSIS; NEUROFILAMENT; NONHUMAN; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN EXPRESSION; SPINAL CORD; TRANSGENIC MOUSE;

EID: 0035873076     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/jneurosci.21-10-03369.2001     Document Type: Article

References (29)

1. Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity
2. Somatic expression of herpes thymidine kinase in mice following injection of a fusion gene into eggs
3. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
4. Neuronal-specific expression of human copper/zinc superoxide dismutase gene in transgenic mice: Animal model of gene dosage effects in Down's syndrome
5. Multiple neuron-specific enhancers in the gene coding for the human neurofilament light chain
6. Isolation of biologically active ribonucleic acid from sources enriched in ribonuclease
7. Glutamate neurotoxicity and diseases of the nervous system
8. A simple test to monitor the motor dysfunction in a transgenic mouse model of amyotrophic lateral sclerosis
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. Transgenic mice with increased Cu/Zn superoxide dismutase activity: Animal model of dosage effects in Down syndrome
11. Superoxide radical and superoxide dismutases
12. Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration
13. Motor neuron degeneration in mice that express a human Cu/Zn superoxide dismutase mutation
14. A distinct pattern of trophic factor expression in myelin-deficient nerves of Trembler mice: Implications for trophic support by Schwann cells
15. Motor neuron degeneration induced by excitotoxin agonists has features in common with those seen in the SOD-1 transgenic mouse model of amyotrophic lateral sclerosis
16. Decreased neurofilament gene expression is an index of selective axonal hypotrophy in aging
17. 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
18. Familial adult motor neuron disease: Amyotrophic lateral sclerosis
19. Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis
20. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
21. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis
22. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
23. Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis
24. Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis
25. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis
26. Knock-out of glutamate transporters reveals a major role for astroglial transport in excitotoxicity and clearance of glutamate
27. Amyotrophic lateral sclerosis. Part 1. Clinical features, pathology, and ethical issues in management
28. A rapid and simple method for introducing specific mutations into any position of DNA leaving all other positions unaltered
29. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria