DOI: 10.1073/pnas.240305897. Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

10.1073/pnas.240305897

Crossref journal-article

Proceedings of the National Academy of Sciences

Proceedings of the National Academy of Sciences (341)

Abstract

Familial amyotrophic lateral sclerosis-linked mutations in copper-zinc superoxide dismutase cause motor neuron death through one or more acquired toxic properties. An early event in the mechanism of toxicity from such mutants is now demonstrated to be activation of caspase-1. Neuronal death, however, follows only after months of chronic caspase-1 activation concomitantly with activation of the executioner caspase-3 as the final step in the toxic cascade. Thus, a common toxicity of mutant SOD1 is a sequential activation of at least two caspases, caspase-1 that acts slowly as a chronic initiator and caspase-3 acting as the final effector of cell death.

Bibliography

Pasinelli, P., Houseweart, M. K., Brown, R. H., & Cleveland, D. W. (2000). Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences, 97(25), 13901â13906.

Authors 4

Piera Pasinelli (first)
Megan K. Houseweart (additional)
Robert H. Brown (additional)
Don W. Cleveland (additional)

References 35 Referenced 277

10.1038/362059a0
10.1016/S0896-6273(00)81108-3
10.1038/ng0596-43
10.1126/science.8209258
10.1016/0896-6273(95)90259-7
10.1016/S0896-6273(00)80272-X
10.1038/378342a0
10.1146/annurev.cellbio.15.1.269
10.1101/gad.12.11.1551
10.1073/pnas.92.7.3024
10.1523/JNEUROSCI.17-22-08756.1997
10.1097/00005072-199705000-00008
10.1016/0896-6273(95)90287-2
J Jordan, G Ghadge, J Prehn, P Toth, R Roos, R Miller Mol Pharmacol 47, 1095–1100 (1995). / Mol Pharmacol by Jordan J (1995)
10.1126/science.277.5325.559
10.1038/40299
10.1126/science.288.5464.335
10.1046/j.1471-4159.1999.0732460.x
10.1002/ana.410400307
10.1111/j.1600-0404.1997.tb00302.x
10.1073/pnas.95.26.15763
10.1038/12381
10.1038/376037a0
10.1074/jbc.271.30.17601
10.1523/JNEUROSCI.18-10-03659.1998
10.1007/s004010050650
10.1023/A:1020605112755
L I Brujin, T L Williamson, M W Becher, D L Price, D W Cleveland Amyotrophic Lateral Sclerosis, eds R H Brown, M Swash, V Meininger (Dunitz, London), pp. 341–362 (1999). / Amyotrophic Lateral Sclerosis by Brujin L I (1999)
10.1038/384368a0
10.1006/geno.1996.4548
10.1002/ana.410380114
10.1038/12227
10.1038/sj.cdd.4400589
10.1038/380723a0
S Kato, H Hayashi, K Nakashima, E Nanba, M Kato, A Hirano, I Nakano, K Asayama, E Ohama Am J Pathol 151, 611–620 (1997). / Am J Pathol by Kato S (1997)

Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 10:44 a.m.)
Deposited	3 years, 4 months ago (April 13, 2022, 9:26 a.m.)
Indexed	1 day, 2 hours ago (Sept. 3, 2025, 6:08 a.m.)
Issued	24 years, 9 months ago (Nov. 28, 2000)
Published	24 years, 9 months ago (Nov. 28, 2000)
Published Online	24 years, 9 months ago (Nov. 28, 2000)
Published Print	24 years, 8 months ago (Dec. 5, 2000)

Funders 0

None

BibTeX

@article{Pasinelli_2000, title={Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis}, volume={97}, ISSN={1091-6490}, url={http://dx.doi.org/10.1073/pnas.240305897}, DOI={10.1073/pnas.240305897}, number={25}, journal={Proceedings of the National Academy of Sciences}, publisher={Proceedings of the National Academy of Sciences}, author={Pasinelli, Piera and Houseweart, Megan K. and Brown, Robert H. and Cleveland, Don W.}, year={2000}, month=nov, pages={13901–13906} }

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