Mitochondrial changes in motor neurons of homozygotes of leucine 126 TT deletion SOD1 transgenic mice

Neuropathology

Volumn 28, Issue 3, 2008, Pages 269-276

  Doi, Koji ^a   Nakano, Toshiya ^a   Kitayama, Michio ^a   Watanabe, Yasuhiro ^a   Yasui, Kenichi ^a   Fukada, Yasuyo ^a   Morino, Shinichi ^b   Kaidoh, Toshiyuki ^a   Nakashima, Kenji ^a   Inoué, Takao ^a  

^a TOTTORI UNIVERSITY   (Japan)

^b TOTTORI UNIVERSITY   (Japan)

Author keywords

Amyotrophic lateral sclerosis (ALS); Leucine 126 TT deletion; Mitochondria; Superoxide dismutase 1 (SOD1); Transgenic mice

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; EPITOPE; GOLD; LEUCINE; SIGNAL PEPTIDE; THYMINE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CARBOXY TERMINAL SEQUENCE; CONTROLLED STUDY; CYTOPLASM; DISEASE SEVERITY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; GENE DELETION; HISTOPATHOLOGY; HOMOZYGOTE; IMMUNOREACTIVITY; MITOCHONDRIAL MEMBRANE; MOTONEURON; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; SPINAL CORD; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; DISEASE MODELS, ANIMAL; HOMOZYGOTE; HUMANS; LEUCINE; MICE; MICE, TRANSGENIC; MICROSCOPY, IMMUNOELECTRON; MITOCHONDRIA; MOTOR NEURONS; PEPTIDES; SEQUENCE DELETION; SUPEROXIDE DISMUTASE;

EID: 43049120643     PISSN: 09196544     EISSN: 14401789     Source Type: Journal    
DOI: 10.1111/j.1440-1789.2007.00876.x     Document Type: Article

References (27)

1. Brown RH Jr. Amyotrophic lateral sclerosis recent insights from genetics and transgenic mice. Cell 1995 80 : 687 692.
2. Nagai M, Aoki M, Miyoshi I et al. Rats expressing human cytosolic Copper-Zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. J Neurosci 2001 23 : 9246 9254.
3. Rosen DR, Siddique T, Patterson D et al. Mutations in Cu/Zn superoxide dismutase are associated with familial amyotrophic lateral sclerosis. Nature 1993 362 : 59 62.
4. Takahashi K, Nakamura H, Okada E. Hereditary amyotrophic lateral sclerosis: histochemical and electron microscopic study of hyaline inclusions in motor neurons. Arch Neurol 1972 27 : 292 299.
5. Watanabe Y, Yasui K, Nakano T et al. Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification. Mol Brain Res 2005 135 : 12 20.
6. Dal Canto MC, Gurney ME. 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). Brain Res 1995 676 : 25 40.
7. Morrison BM, Janssen WG, Gordon JW, Morrison JH. Time course of neuropathology in the spinal cord of H86R superoxide dismutase transgenic mice. J Comp Neurol 1998 391 : 64 77.
8. Shibata N. Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation. Neuropathol 2001 21 : 82 92.
9. Wong PC, Pardo CA, Borchelt DR et al. An adverse property of familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 1995 14 : 1105 1116.
10. Higgins CMJ, Cheolwha J, Xu Z. ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci 2003 4 : 16 29.
11. Jaarsma D, Haasdijk ED, Grashorn JAC et al. Human Cu/Zn: superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motor neuron disease in mice expressing a familial lateral sclerosis mutant SOD1. Neurobiol Dis 2000 7 : 623 643.
12. Kong J, Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J Neurosci 1998 18 : 3241 3250.
13. Liu J, Lillo C, Jonsson PA et al. Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 2004 43 : 5 17.
14. Sasaki S, Warita H, Murakami T, Abe K, Iwata M. Ultrastructural study of mitochondria in the spinal cord of transgenic mice with a G93A mutant SOD1 gene. Acta Neuropathl 2004 107 : 461 474.
15. Stieber A, Gonatas JO, Gonatas NK. Aggregates of mutant protein appear progressively in dendrites, in periaxonal processes of oligodendrocytes, and in neuronal and astrocytic perikarya of mice expressing the SOD1G93A mutation of familial amyotrophic lateral sclerosis. J Neurol Sci 2000 177 : 114 123.
16. Mourelatos Z, Gonatas NK, Stieber A, Gurney ME, Canto MCD. The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu, Zn superoxide dismutase becomes fragmented in early, preclinical stage of the disease. Proc Natl Acad Sci USA 1996 93 : 5472 5477.
17. Sasaki S, Warita H, Abe K, Iwata M. Impairment of axonal transport in the axon hillock and the initial segment of anterior horn neurons in transgenic mice with a G93A mutant SOD1 gene. Acta Neuropathol 2005 110 : 48 56.
18. Wang J, Gonzales V, Coonfield M et al. Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol Dis 2002 10 : 128 138.
19. Bendotti C, Calvaresi N, Chiveri L et al. Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity. J Neurol Sci 2001 191 : 25 33.
20. Chiu. AY, Zhai P, Canto MCD et al. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol Cell Neurosci 1995 6 : 349 362.
21. Xu Z, Jung Ch, Higgins C, Levine J, Kong J. Mitochondrial degeneration in amyotrophic lateral sclerosis. J Bioenerg Biomembr 2004 36 : 395 399.
22. Pasinelli P, Belford ME, Lennon N et al. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron 2004 43 : 19 30.
23. Estevesz AG, Crow JP, Sampson JB et al. Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase. Science 1999 286 : 2498 2500.
24. Manfredi G, Xu Z. Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion 2005 5 : 77 87.
25. Morrison BM, Janssen WG, Gordon JW, Morrison JH. Light and electron microscopic distribution of the AMPA receptor subunit, GluR2, in the spinal cord of control and G86R mutant superoxide dismutase transgenic mice. J Comp Neurol 1998 395 : 523 534.
26. Jaarsma D, Rognoni F, Duijn WV, Verspaget HW, Haasdijk ED, Hoistege JC. CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations. Acta Neuropathl 2001 102 : 293 305.
27. Sumi H, Nagano S, Fujimura H, Kato S, Sakoda S. Inverse correlation between the formation of mitochondria-derived vacuoles and Lewy-body-like hyaline inclusions in G93A superoxide-dismutase-transgenic mice. Acta Neuropathol (Berl) 2006 112 : 52 63.