Vacuolization correlates with spin-spin relaxation time in motor brainstem nuclei and behavioural tests in the transgenic G93A-SOD1 mouse model of ALS

European Journal of Neuroscience

Volumn 26, Issue 7, 2007, Pages 1895-1901

  Bucher, Selina ^a   Braunstein, Kerstin E ^a   Niessen, Heiko G ^b   Kaulisch, Thomas ^b   Neumaier, Michael ^b   Boeckers, Tobias M ^a   Stiller, Detlef ^b   Ludolph, Albert C ^a  

^a UNIVERSITY OF ULM   (Germany)

^b BOEHRINGER INGELHEIM PHARMA GMBH AND CO KG   (Germany)

Author keywords

Amyotrophic lateral sclerosis; Open field behavioural test; Superoxide dismutase 1; T2 maps; Transverse relaxation time T2

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL BEHAVIOR; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN STEM; CELL VACUOLE; CONTROLLED STUDY; CORRELATION ANALYSIS; DISEASE COURSE; FOLLOW UP; HISTOPATHOLOGY; IN VIVO STUDY; LOCOMOTION; MALE; MOTONEURON NUCLEUS; MOTOR PERFORMANCE; MOUSE; NERVE CELL DEGENERATION; NEUROPATHOLOGY; NON INVASIVE PROCEDURE; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; OPEN FIELD BEHAVIOR; PRIORITY JOURNAL; RELAXATION TIME; TRANSGENIC MOUSE;

AGE FACTORS; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; ANIMALS, NEWBORN; BEHAVIOR, ANIMAL; BRAIN STEM; DENDRITIC SPINES; DISEASE MODELS, ANIMAL; EXPLORATORY BEHAVIOR; HUMANS; MAGNETIC RESONANCE IMAGING; MALE; MICE; MICE, TRANSGENIC; MOTOR NEURONS; SUPEROXIDE DISMUTASE;

EID: 34848912811     PISSN: 0953816X     EISSN: 14609568     Source Type: Journal    
DOI: 10.1111/j.1460-9568.2007.05831.x     Document Type: Article

References (31)

1. Angenstein, F., Niessen, H.G., Goldschmidt, J., Vielhaber, S., Ludolph, A.C. Scheich, H. (2004) Age-dependent changes in MRI of motor brain stem nuclei in a mouse model of ALS. Neuroreport, 15, 2271 2274.
2. Brown, R.H. (1995) Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function. Curr. Opin. Neurobiol., 5, 841 846.
3. Bruijn, L.I., Miller, T.M. Cleveland, D.W. (2004) Unraveling the mechanisms involved in motor neuron degeneration in ALS. Ann. Rev. Neurosci., 27, 723 749.
4. Cleveland, D.W. Rothstein, J.D. (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat. Rev. Neurosci., 2, 806 819.
5. Dobrowolny, G., Giacinti, C., Pelosi, L., Nicoletti, C., Winn, N., Barberi, L., Molinaro, M., Rosenthal, N. Musaro, A. (2005) Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model. J. Cell. Biol., 168, 193 199.
6. Gurney, M.E. (1997) The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies. J. Neurol. Sci., 152 (Suppl. 1 S67 S73.
7. Gurney, M.E., Pu, H., Chiu, A.Y., Dal Canto, M.C., Polchow, C.Y., Alexander, D.D., Caliendo, J., Hentati, A., Kwon, Y.W., Deng, H.X., Chen, W.J., Zhai, P., Sufit, R.L. Siddique, T. (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science, 264, 1772 1775.
8. Hafezparast, M., Klocke, R., Ruhrberg, C., Marquardt, A., Ahmad-Annuar, A., Bowen, S., Lalli, G., Witherden, A.S., Hummerich, H., Nicholson, S., Morgan, P.J., Oozageer, R., Priestley, J.V., Averill, S., King, V.R., Ball, S., Peters, J., Toda, T., Yamamoto, A., Hiraoka, Y., Augustin, M., Korthaus, D., Wattler, S., Wabnitz, P., Dickneite, C., Lampel, S., Boehme, F., Peraus, G., Popp, A., Rudelius, M., Schlegel, J., Fuchs, H., Hrabe de Angelis, M., Schiavo, G., Shima, D.T., Russ, A.P., Stumm, G., Martin, J.E. Fisher, E.M. (2003) Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science, 300, 808 812.
9. Harris, E.D. (1998) Amyotrophic lateral sclerosis: a lesson in deficiency diseases. Nutr. Rev., 56, 81 84.
10. Heiman-Patterson, T.D., Deitch, J.S., Blankenhorn, E.P., Erwin, K.L., Perreault, M.J., Alexander, B.K., Byers, N., Toman, I. Alexander, G.M. (2005) Background and gender effects on survival in the TgN (SOD1-G93A), 1Gur mouse model of ALS. J. Neurosci., 236, 1 7.
11. Hennig, J., Nauerth, A. Friedburg, H. (1986) RARE imaging: a fast imaging method for clinical MR. Magn. Reson. Med., 3, 823 833.
12. Higgins, C.M., Jung, C. Xu, Z. (2003) ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci., 4, 16.
13. Jaarsma, D., Haasdijk, E.D., Grashorn, J.A., Hawkins, R., van Duijn, W., Verspaget, H.W., London, J. Holstege, J.C. (2000) Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motorneuron death and accelerates motorneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1. Neurobiol. Dis., 7, 623 643.
14. Kaspar, B.K., Llado, J., Sherkat, N., Rothstein, J.D. Gage, F.H. (2003) Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model. Science, 301, 839 842.
15. Kieran, D., Hafezparast, M., Bohnert, S., Dick, J.R., Martin, J., Schiavo, G., Fisher, E.M. Greensmith, L. (2005) A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J. Cell Biol., 169, 561 567.
16. Kong, J. Xu, Z. (1998) Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J. Neurosci., 18, 3241 3250.
17. Mattiazzi, M., D'Aurelio, M., Gajewski, C.D., Martushova, K., Kiaei, M., Beal, M.F. Manfredi, G. (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J. Biol. Chem., 277, 29626 29633.
18. Mulder, D.W., Kurland, L.T., Offord, K.P. Beard, C.M. (1986) Familial adult motor neuron disease: amyotrophic lateral sclerosis. Neurology, 36, 511 517.
19. 2 relaxation time and apparent diffusion coefficient. Exp. Neurol., 201, 293 300.
20. Niessen, H.G., Debska-Vielhaber, G., Sander, K., Angenstein, F., Ludolph, A.C., Hilfert, L., Willker, W., Leibfritz, D., Heinze, H.J., Kunz, W.S. Vielhaber, S. (2007) Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis. Eur. J. Neurosci., 25, 1669 1677.
21. Paxinos, G. Franklin, K.B.J. (2000) The Mouse Brain in Stereotaxic Coordinates, 2nd Edn. Academic Press, San Diego.
22. Ripps, M.E., Huntley, G.W., Hof, P.R., Morrison, J.H. Gordon, J.W. (1995) Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc. Natl Acad. Sci. USA, 92, 689 693.
23. Rosen, D.R., Siddique, T., Patterson, D., Figlewicz, D.A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J.P., Deng, H.X., Rahmani, Z., Krizus, A., McKennayasek, D., Cayabyab, A., Gaston, S.M., Berger, R., Tanzi, R.E., Halperin, J.J., Herzfeldt, B., Vandenbergh, R., Hung, W.Y., Bird, T., Deng, G., Mulder, D.W., Smyth, C., Laing, N.G., Soriano, E., Pericakvance, M.A., Haines, J., Rouleau, G.A., Gusella, J.S., Horvitz, H.R. Brown, R.H. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 362, 59 62.
24. Rowland, L.P. Shneider, N.A. (2001) Amyotrophic lateral sclerosis. N. Engl. J. Med., 344, 1688 1700.
25. Schutz, B., Reimann, J., Dumitrescu-Ozimek, L., Kappes-Horn, K., Landreth, G.E., Schurmann, B., Zimmer, A. Heneka, M.T. (2005) The oral antidiabetic pioglitazone protects from neurodegeneration and amyotrophic lateral sclerosis-like symptoms in superoxide dismutase-G93A transgenic mice. J. Neurosci., 25, 7805 7812.
26. Shaw, P.J. (1999) Motor neurone disease. BMJ, 318, 1118 1121.
27. Talbot, K. (2002) Motor neurone disease. Postgrad. Med. J., 78, 513 519.
28. Teuchert, M., Fischer, D., Schwalenstoecker, B., Habisch, H.J., Bockers, T.M. Ludolph, A.C. (2006) A dynein mutation attenuates motor neuron degeneration in SOD1 (G93A) mice. Exp. Neurol., 198, 271 274.
29. Weydt, P., Hong, S.Y., Kliot, M. Moller, T. (2003) Assessing disease onset and progression in the SOD1 mouse model of ALS. Neuroreport, 14, 1051 1054.
30. Wong, P.C., Pardo, C.A., Borchelt, D.R., Lee, M.K., Copeland, N.G., Jenkins, N.A., Sisodia, S.S., Cleveland, D.W. Price, D.L. (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron, 14, 1105 1116.
31. Zang, D.W., Yang, Q., Wang, H.X., Egan, G., Lopes, E.C. Cheema, S.S. (2004) Magnetic resonance imaging reveals neuronal degeneration in the brainstem of the superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis. Eur. J. Neurosci., 20, 1745 1751.