Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

Proceedings of the National Academy of Sciences of the United States of America

Volumn 103, Issue 18, 2006, Pages 7142-7147

  Deng, Han Xiang ^a   Shi, Yong ^a   Furukawa, Yoshiaki ^a   Zhai, Hong ^a   Fu, Ronggen ^a   Liu, Erdong ^a   Gorrie, George H ^a   Khan, Mohammad S ^a   Hung, Wu Yen ^a   Bigio, Eileen H ^a   Lukas, Thomas ^a   Dal Canto, Mauro C ^a   O'Halloran, Thomas V ^a   Siddique, Teepu ^a  

^a NORTHWESTERN UNIVERSITY   (United States)

Author keywords

Crosslinked; Disulfide bonds; Neurodegeneration; Oxidation; Protein aggregation

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; CYSTEINE; DIMER; FREE RADICAL;

AGING; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL TISSUE; ARTICLE; BRAIN MITOCHONDRION; CONTROLLED STUDY; DEGENERATIVE DISEASE; DISEASE EXACERBATION; DISULFIDE BOND; FAMILIAL DISEASE; GENE; GENE MUTATION; MOUSE; MUTANT; NONHUMAN; OXIDATION REDUCTION REACTION; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN CROSS LINKING; SOD1 GENE; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; HUMANS; MICE; MICE, TRANSGENIC; MITOCHONDRIA; MUTATION; PHENOTYPE; PROTEIN CONFORMATION; SPINAL CORD; SUPEROXIDE DISMUTASE; SURVIVAL RATE;

MUS MUSCULUS;

EID: 33646466296     PISSN: 00278424     EISSN: None     Source Type: Journal    
DOI: 10.1073/pnas.0602046103     Document Type: Article

References (39)

1. Hughes, J. T. (1982) Adv. Neurol. 36, 61-74.
2. Lacomblez, L., Bensimon, G., Leigh, P. N., Guillet, P. & Meininger, V. (1996) Lancet 347, 1425-1431.
3. 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., et al. (1993) Nature 362, 59-62.
4. Deng, H.-X., Hentati, A., Tainer, J. A., Iqbal, Z., Cayabyab, A., Hung, W. Y., Getzoff, E. D., Hu, P., Herzfeldt, B., Roos, R. P., et al. (1993) Science 261, 1047-1051.
5. 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., et al. (1994) Science 264, 1772-1775.
6. Reaume, A. G., Elliott, J. L., Hoffman, E. K., Kowall, N. W., Ferrante, R. J., Siwek, D. F., Wilcox, H. M., Flood, D. G., Beal, M. F., Brown, R. H., Jr, et al. (1996) Nat. Genet. 13, 43-47.
7. Liochev, S. I. & Fridovich I. (2003) Free Radical Biol. Med. 34, 1383-1389.
8. Dal Canto, M. C. & Gurney, M. E. (1995) Brain Res. 676, 25-40.
9. Shibata, N., Hirano, A., Kobayashi, M., Siddique, T., Deng, H.-X., Hung, W. Y., Kato, T. & Asayama, K. (1996) J. Neuropathol. Exp. Neurol. 55, 481-490.
10. Bruijn, L. I., Houseweart, M. K., Kato, S., Anderson, K. L., Anderson, S. D., Ohama, E., Reaume, A. G., Scott, R. W. & Cleveland, D. W. (1998) Science 281, 1851-1854.
11. Cleveland, D. W. & Liu, J. (2000) Nat. Med. 6, 1320-1321.
12. Bruijn, L. I., Miller, T. M. & Cleveland, D. W. (2004) Annu. Rev. Neurosci. 27, 723-749.
13. Sherman, M. Y. & Goldberg, A. L. (2001) Neuron 29, 15-32.
14. Deng, H.-X., Fu, R. & Siddique, T. (1998) Am. J. Hum. Genet. Suppl. 63, A2069.
15. Jaarsma, D., Haasdijk, E. D., Grashorn, J. A., Hawkins, R., Van Duijn, W., Verspaget, H. W., London, J. & Holstege, J. D. (2000) Neurobiol. Dis. 7, 623-643.
16. Zu, J. S., Deng, H.-X., Lo, T. P., Mitsumoto, H., Ahmed, M. S., Hung, W.-Y., Cai, Z. J., Tainer, J. A. & Siddique, T. (1997) Neurogenetics 1, 65-71.
17. Deng, H.-X., Fu, R., Zhai, H. & Siddique, T. (1999) Am. J. Hum. Genet. Suppl. 65, A1636.
18. Jonsson, P. A., Ernhill, K., Andersen, P. M., Bergemalm, D., Brannstrom, T., Gredal, O., Nilsson, P. & Marklund, S. L. (2004) Brain 127, 73-88.
19. Watanabe, Y., Yasui, K., Nakano, T., Doi, K., Fukada, Y., Kitayama, M., Ishimoto, M., Kurihara, S., Kawashima, M., Fukuda, H., et al. (2005) Brain Res. Mol. Brain Res. 135, 12-20.
20. Wang, J., Xu, G., Li, H., Gonzales, V., Fromholt, D., Karch, C., Copeland, N. G., Jenkins, N. A. & Borchelt, D. R. (2005) Hum. Mol. Genet. 14, 2335-2347.
21. Weisiger, R. A. & Fridovich, I. (1973) J. Biol. Chem. 248, 3582-3592.
22. Chang, L. Y., Slot, J. W., Geuze, H. J. & Crapo, J. D. (1988) J. Cell Biol. 107, 2169-2179.
23. Higgins, C. M., Jung, C. & Xu, Z. (2003) BMC Neurosci. 4, 16.
24. Pasinelli, P., Belford, M. E., Lennon, N., Bacskai, B. J., Hyman, B. T., Trotti, D. & Brown, R. H., Jr. (2004) Neuron 43, 19-30.
25. Liu, J., Lillo, C., Jonsson, P. A., Vande Velde, C., Ward, C. M., Miller, T. M., Subramaniam, J. R., Rothstein, J. D., Marklund, S. L., Andersen, P. M., et al. (2004) Neuron 43, 5-17.
26. Vijayvergiya, C., Beal, M. F., Buck, J. & Manfredi, G. (2005) J. Neurosci. 25, 2463-2470.
27. Furukawa, Y., Torres, A. S. & O'Halloran, T. V. (2004) EMBO J. 23, 2872-2881.
28. Furukawa, Y. & O'Halloran, T. V. (2005) J. Biol. Chem. 280, 17266-17274.
29. Lepock, J. R., Frey, H. E. & Hallewell, R. A. (1990) J. Biol. Chem. 265, 21612-21618.
30. Fukada, K., Nagano, S., Satoh, M., Tohyama, C., Nakanishi, T., Shimizu, A., Yanagihara, T. & Sakoda, S. (2001) Eur. J. Neurosci. 14, 2032-2036.
31. Rakhit, R., Crow, J. P., Lepock, J. R., Kondejewski, L. H., Cashman, N. R. & Chakrabartty, A. (2004) J. Biol. Chem. 279, 15499-15504.
32. Field, L. S., Furukawa, Y., O'Halloran, T. V. & Culotta, V. C. (2003) J. Biol. Chem. 278, 28052-28059.
33. Jonsson, P. A., Graffmo, K. S., Andersen, P. M., Brannstrom, T., Linderberg, M., Oliverberg, M. & Marklund, S. L. (2006) Brain 129, 451-464.
34. Tiwari, A. & Hayward, L. J. (2003) J. Biol. Chem. 278, 5984-5992.
35. Taylor, J. P., Hardy, J. & Fischbeck, K. H. (2002) Science 296, 1991-1995.
36. Butterfield, D. A. & Kanski, J. (2001) Mech. Ageing Dev. 122, 945-962.
37. Beal, M. F. (2002) Free Radical Biol. Med. 32, 797-803.
38. Lee, S. & Eisenberg, D. (2003) Nat. Struct. Biol. 10, 725-730.
39. Furukawa, Y., Fu, R., Deng, H.-X., Siddique, T. & O'Halloran, T. V. (2006) Proc. Natl. Acad. Sci. USA, in press.