Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis

Nature Medicine

Volumn 5, Issue 3, 1999, Pages 347-350

  Klivenyi, Peter ^a   Ferrante, Robert J ^b,c   Matthews, Russell T ^a   Bogdanov, Mikhail B ^a   Klein, Autumn M ^b,c   Andreassen, Ole A ^a   Mueller, Gerald ^a   Wermer, Marieke ^a   Kaddurah Daouk, Rima ^d   Beal, M Flint ^a,e  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c DEPARTMENT OF VETERANS AFFAIRS   (United States)

^d Avicena Group   (United States)

^e WEILL CORNELL MEDICAL COLLEGE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

3 NITROTYROSINE; CREATINE; CREATINE KINASE; MITOCHONDRIAL ENZYME; SUPEROXIDE DISMUTASE;

ADOLESCENT; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; BRAIN PROTECTION; CONTROLLED STUDY; DIET SUPPLEMENTATION; DOSE RESPONSE; DRUG EFFECT; MOTOR PERFORMANCE; NONHUMAN; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL; SURVIVAL; TRANSGENIC MOUSE;

ALANINE; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; CREATINE; DISEASE MODELS, ANIMAL; GLYCINE; HUMANS; MICE; MICE, TRANSGENIC; MOTOR ACTIVITY; MUSCLE, SKELETAL; NEURONS; NEUROPROTECTIVE AGENTS; SUPEROXIDE DISMUTASE; TYROSINE; TYROSINE 3-MONOOXYGENASE;

ANIMALIA; MUS MUSCULUS;

EID: 0033051815     PISSN: 10788956     EISSN: None     Source Type: Journal    
DOI: 10.1038/6568     Document Type: Article

References (23)

1. Wong, P.C. et al. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14, 1105-1116 (1995).
2. Gurney, M.E. et al. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264, 1772-1775 (1994).
3. 2+ concentration in transfected neuroblastoma SH-SY5Y cells. FEBS Lett. 414, 365-368 (1997).
4. Hemmer, W. & Wallimann, T. Functional aspects of creatine kinase in brain. Dev. Neurosci. 15, 249-260 (1993).
5. O'Gorman, E., Beutner, C., Wallimann, T. & Brdiczka, D. Differential effects of creatine depletion on the regulation of enzyme activities and on creatine-stimulated mitochondrial respiration in skeletal muscle, heart, and brain. Biochim. Biophys. Acta 1276, 161-170 (1996).
6. Gurney, M.E. et al. Antioxidants and inhibitors of glutamatergic transmission have therapeutic benefit in a transgenic model of familial amyotrophic lateral sclerosis. Am. Neurol. 39, 147-157 (1996).
7. Dugan, L.L. et al. Carboxyfullerenes as neuroprotective agents. Proc. Natl. Acad. Sci. USA 94, 9434-9439 (1997).
8. Hottinger, A.F., Fine, E.G., Gurney, M.E., Zurn, A.D. & Aebischer, P. The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic moouse model of familial amyotrophic lateral sclerosis. Eur. J. Neurosci. 9, 1548-1551 (1997).
9. Kostic, V. et al. Midbrain dopaminergic neuronal degeneration in a transgenic mouse model of familial amyotrophic lateral sclerosis. Ann. Neurol. 41, 497-504 (1997).
10. Ferrante, R.J. et al. Increased 3-nitrotyrosine and oxidative damage in mice with a human Cu, Zn superoxide dismutase mutation. Ann. Neurol. 42, 326-334 (1997).
11. Bogdanov, M.B., Ramos, L.E., Xu, X. & Beal, M.F. Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis. J. Neurochem. 71, 1321-1324 (1998).
12. Sasaki, S., Maruyama, S., Yamane, K., Sakuma, H. & Takeishi, M. Ultrastructure of swollen proximal axons of anterior horn neurons in motor neuron disease. J. Neurol. Sci. 97, 233-240 (1990).
13. Siklos, L. et al. Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis. Ann. Neurol. 39, 203-219 (1996).
14. White, R.J. & Reynolds, I.J. Mitochondrial depolarization in glutamate-stimulated neurons: an early signal specific to excitotoxin exposure. J. Neurosci. 16, 5688-5697 (1996).
15. O'Gorman, E. et al. The role of creatine kinase inhibition of mitochondrial permeability transition. FEBS Lett. 414, 253-257 (1997).
16. Sauter, A. & Rudin, M. Determination of creatine kinase parameters in rat brain by NMR magnetization transfer: correlation with brain function. J. Biol. Chem. 268, 13166-13171 (1993).
17. 31P NMR magnetization transfer. J. Cereb. Blood Flow Metab. 14, 1070-1077 (1994).
18. Xu, C.J. et al. Phosphocrealine-dependent glutamate uptake by synaptic vesicles. J. Biol. Chem. 271, 13435-13440 (1996).
19. Rothstein, J.D., M., V.K., Levey, A.I., Martin, L. & Kuncl, R.W. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis. Ann. Neurol. 38, 73-84 (1995).
20. Sipila, I., Rapola, J., Simell, O. & Vannas, A. Supplementary creatine as a treatment for gyrate atrophy of the choroid and retina. N. Engl. J. Med. 304, 867-870 (1981).
21. Blanchard, V., Raisman-Vozari, R., Vyas, S., Michel, P.P., Javoy-Agid, F. & Uhl, G. Differential expression of tyrosine hydroxylase and membrane dopamine transporter genes in subpopulations of dopaminergic neurons of rat mesencephalon. Molec. Brain Res. 22, 29-40 (1994).
22. Matthews, R.T. et al. Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease. J. Neurosci. 18, 156-163 (1998).
23. Bogdanov, M.B., Ramos, L.E., Xu, X. & Beal, M.F. Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis. J. Neurochem. 71, 1321-1324 (1998).