Mitochondrial Degeneration in Amyotrophic Lateral Sclerosis
Tóm tắt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that causes motor neuron degeneration, progressive skeletal muscle atrophy, paralysis, and death. To understand the mechanism of motor neuron degeneration, we have analyzed the clinical disease progression and the pathological changes in a transgenic mouse model for ALS. We found massive mitochondrial vacuolation at the onset of disease. By detailed morphological observations, we have determined that this mitochondrial vacuolation is developed from expansion of mitochondrial intermembrane space and extension of the outer membrane and involves peroxisomes. Lysosomes do not actively participate at all stages of this vacuolation. We conclude that this mitochondrial vacuolation is neither classical mitochondrial permeability transition nor autophagic vacuolation. Thus, this appears to be a new form of mitochondrial vacuolation and we term this as mitochondrial vacuolation by intermembrane space expansion or MVISE.
Tài liệu tham khảo
Aggarwal, A., and Nicholson, G. (2002). J. Neurol. Neurosurg. Psychiatry. 73, 199–201.
Andreassen, O. A., Ferrante, R. J., Klivenyi, P., Klein, A. M., Dedeoglu, A., Albers, D. S., Kowall, N. W., and Beal, M. F. (2001). Exp. Neurol. 168, 356–363.
Andreassen, O. A., Ferrante, R. J., Klivenyi, P., Klein, A. M., Shinobu, L. A., Epstein, C. J., and Beal, M. F. (2000). Ann. Neurol. 47, 447–455.
Batulan, Z., Shinder, G. A., Minotti, S., He, B. P., Doroudchi, M. M., Nalbantoglu, J., Strong, M. J., and Durham, H. D. (2003). J. Neurosci. 23, 5789–5798.
Beal, M. F. (1992). Ann. Neurol. 31, 119–130.
Bendotti, C., Calvaresi, N., Chiveri, L., Prelle, A., Moggio, M., Braga, M., Silani, V., and De Biasi, S. (2001). J. Neurol. Sci. 191,25–33.
Bittigau, P., and Ikonomidou, C. (1997). J. Child Neurol. 12, 471–485.
Bruening, W., Roy, J., Giasson, B., Figlewicz, D. A., Mushynski, W. E., and Durham, H. D. (1999). J. Neurochem. 72, 693–699.
Bruijn, L. I., Becher, M. W., Lee, M. K., Anderson, K. L., Jenkins, N.A., Copeland, N. G., Sisodia, S. S., Rothstein, J. D., Borchelt, D. R., Price, D. L., and Cleveland, D. W. (1997). Neuron. 18, 327–338.
Bruijn, L. I., Houseweart, M. K., Kato, S., Anderson, K. L., Anderson, S. D., Ohama, E., Reaume, A. G., Scott, R. W., and Cleveland, D. W. (1998). Science 281, 1851–1854.
Carpenter, S. (1968). Neurol. 18, 841–851.
Carri, M. T., Ferri, A., Battistoni, A., Famhy, L., Cabbianelli, R., Poccia, F., and Rotilio, G. (1997). FEBS Lett. 414, 365–368.
Cleveland, D. W., and Rothstein, J. D. (2001). Nat. Rev. Neurosci. 2, 806–819.
Cox, P. A., Banack, S. A., and Murch, S. J. (2003). Proc. Natl. Acad. Sci. U.S.A. 100, 13380–13383.
Dal Canto, M. C., and Gurney, M. E. (1995). Brain Res. 676,25–40.
De la Rua-Domenech, R., Mohammed, H. O., Cummings, J. F., Divers, T. J., De Lahunta, A., and Summers, B. A. (1997). Vet. J. 154, 203–213.
Green, D. R., and Evan, G. I. (2002). Cancer Cell 1,19–30.
Guegan, C., and Przedborski, S. (2003). J. Clin. Invest. 111, 153–161.
Guegan, C., Vila, M., Rosoklija, G., Hays, A. P., and Przedborski, S. (2001). J. Neurosci. 21, 6569–6576.
Gurney, M. E. (1994). N. Engl. J. Med. 331, 1721–1722.
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., Zhai, P., Sufit, R. L., and Siddique, T. (1994). Science 264, 1772–1775.
Higgins, C. M., Jung, C., Ding, H., and Xu, Z. (2002). J. Neurosci. 22, RC215.
Higgins, C. M., Jung, C., and Xu, Z. (2003). BMC Neurosci. 4, 16.
Hirano, A. (1991). Adv. Neurol. 56,91–101.
Hirano, A., Donnenfeld, H., Sasaki, S., and Nakano, I. (1984a). J. Neuropath. Exp. Neurol. 43, 461–470.
Hirano, A., Nakano, I., Kurland, L. T., Mulder, D. W., Holley, P. W., and Saccomanno, G. (1984b). J. Neuropath. Exp. Neurol. 43, 471–480.
Ikonomidou, C., Qin Qin, Y., Labruyere, J., and Olney, J. W. (1996). J. Neuropath. Exp. Neurol. 55, 211–224.
Jaarsma, D., Rognoni, F., van Duijn, W., Verspaget, H. W., Haasdijk, E. D., and Holstege, J. C. (2001). Acta Neuropathol. (Berl.) 102, 293–305.
Julien, J. P. (2001). Cell 104, 581–591.
Jung, C., Higgins, C. M., and Xu, Z. (2002). J. Neurochem. 83, 535–545.
Kaal, E. C., Vlug, A. S., Versleijen, M. W., Kuilman, M., Joosten, E. A., and Bar, P. R. (2000). J. Neurochem. 74, 1158–1165.
Kasarskis, E. J., and Winslow, M. (1989). Neurology 39, 1243–1245.
Kong, J., and Xu, Z. (1998). J. Neurosci. 18, 3241–3250.
Kruman, P. W. A., II, Springer, J. E., and Mattson, M. P. (1999). Exp. Neurol. 160,28–39.
Lashuel, H. A., Petre, B. M., Wall, J., Simon, M., Nowak, R. J., Walz, T., and Lansbury, P. T., Jr. (2002). J. Mol. Biol. 322, 1089–1102.
Levine, J. B., Kong, J., Nadler, M., Xu, Z. (1999). Glia 28, 215–224.
Liu, R., Li, B., Flanagan, S. W., Oberley, L. W., Gozal, D., and Qiu, M. (2002). J. Neurochem. 80, 488–500.
Masui, Y., Mozai, T., and Kakehi, K. (1985). J. Neurol. 232,15–19.
Mattiazzi, M., D'Aurelio, M., Gajewski, C. D., Martushova, K., Kiaei, M., Beal, M. F., and Manfredi, G. (2002). J. Biol. Chem. 277, 29626–29633.
Mattson, M. P., and Duan, W. (1999). J. Neurosci. Res. 58, 152–166.
Menzies, F. M., Cookson, M. R., Taylor, R. W., Turnbull, D. M., Chrzanowska-Lightowlers, Z. M., Dong, L., Figlewicz, D. A., and Shaw, P. J. (2002a). Brain 125, 1522–1533.
Menzies, F. M., Ince, P. G., and Shaw, P. J. (2002b). Neurochem. Int. 40, 543–551.
Neupert, W., and Brunner, M. (2002). Nat. Rev. Mol. Cell. Biol. 3, 555–565.
Okado-Matsumoto, A., and Fridovich, I. (2001). J. Biol. Chem. 276, 38388–38393.
Okado-Matsumoto, A., and Fridovich, I. (2002). PNAS 99, 9010–9014.
Pasinelli, P., Houseweart, M. K., Brown, R. H., Jr., and Cleveland, D. W. (2000). Proc. Natl. Acad. Sci. U.S.A. 97, 13901–13906.
Ripps, M. E., Huntley, G. W., Hof, P. R., Morrison, J. H., and Gordon, J. W. (1995). Proc. Natl. Acad. Sci. U.S.A. 92, 689–693.
Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto J., O'Regan J. P., and Deng H. X., et al. (1993). Nature 362,59–62.
Rowland, L. P., and Shneider, N. A. (2001). N. Engl. J. Med. 344, 1688–1700.
Sasaki, S., and Iwata, M. (1999). Neurosci. Lett. 268,29–32.
Sasaki S., Maruyama S., Yamane K., Sakuma H., and Takeishi, M. (1990). J. Neurol. Sci. 97, 233–240.
Shinder, G. A., Lacourse, M.-C., Minotti, S., and Durham, H. D. (2001). J. Biol. Chem. 276, 12791–12796.
Sturtz, L. A., Diekert, K., Jensen, L. T., Lill, R., and Culotta, V. C. (2001). J. Biol. Chem. 276, 38084–38089.
Takeuchi, H., Kobayashi, Y., Ishigaki, S., Doyu, M., and Sobue, G. (2002). J. Biol. Chem. 277, 50966–50972.
Volles, M. J., Lee, S. J., Rochet, J. C., Shtilerman, M. D., Ding, T. T., Kessler, J. C., and Lansbury, P. T., Jr. (2001). Biochemistry 40, 7812–7819.
Wang, J., Xu G., Gonzales, V., Coonfield, M., Fromholt, D., Copeland, N. G., Jenkins, N. A., and Borchelt, D. R. (2002). Neurobiol. Dis. 10, 128–138.
Wiedemann F. R., Manfredi G., Mawrin C., Beal M. F., and Schon, E. A. (2002). J. Neurochem. 80, 616–625.
Wong, P. C., Pardo, C. A., Borchelt, D. R., Lee, M. K., Copeland, N. G., Jenkins, N. A., Sisodia, S. S., Cleveland, D. W., and Price, D. L. (1995). Neuron 14, 1105–1116.
Young, J. C., Hoogenraad, N. J., and Hartl, F. U. (2003). Cell 112,41–50.