Abstract：
:Abstract Dorfin is a RING-finger type ubiquitin ligase for mutant superoxide dismutase 1 (SOD1) that enhances its degradation. Mutant SOD1s cause familial amyotrophic lateral sclerosis (FALS) through the gain of unelucidated toxic properties. We previously showed that the accumulation of mutant SOD1 in the mitochondria triggered the release of cytochrome c, followed by the activation of the caspase cascade and induction of neuronal cell death. In the present study, therefore, we investigated whether Dorfin can modulate the level of mutant SOD1 in the mitochondria and subsequent caspase activation. We showed that Dorfin significantly reduced the amount of mutant SOD1 in the mitochondria, the release of cytochrome c and the activation of the following caspase cascade, thereby preventing eventual neuronal cell death in a neuronal cell model of FALS. These results suggest that reducing the accumulation of mutant SOD1 in the mitochondria may be a new therapeutic strategy for mutant SOD1-associated FALS, and that Dorfin may play a significant role in this.
journal_name
J Neurochemjournal_title
Journal of neurochemistryauthors
Takeuchi H,Niwa J,Hishikawa N,Ishigaki S,Tanaka F,Doyu M,Sobue Gdoi
10.1046/j.1471-4159.2003.02289.xkeywords:
subject
Has Abstractpub_date
2004-04-01 00:00:00pages
64-72issue
1eissn
0022-3042issn
1471-4159pii
JNC2289journal_volume
89pub_type
杂志文章abstract：:The functions of the antiapoptotic proteins Bcl-2 and Bcl-xL were examined in glioblastoma cells. Expression of both Bcl-2 and Bcl-xL were found to be elevated in protein lysates from seven early passage cell lines derived from human glioblastoma tumors compared with non-neoplastic glial cells. Down-regulation of both...
journal_title：Journal of neurochemistry
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pub_type： 杂志文章
doi：10.1046/j.1471-4159.1996.67020717.x
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pub_type： 杂志文章
doi：10.1111/j.1471-4159.1992.tb10128.x
更新日期：1992-12-01 00:00:00
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journal_title：Journal of neurochemistry
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pub_type： 杂志文章
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更新日期：2009-05-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1983.tb11297.x
更新日期：1983-02-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.1999.0732321.x
更新日期：1999-12-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1989.tb07366.x
更新日期：1989-08-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.2005.03179.x
更新日期：2005-07-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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更新日期：2014-06-01 00:00:00
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doi：10.1046/j.1471-4159.2000.0741000.x
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doi：10.1111/j.1471-4159.1993.tb03634.x
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journal_title：Journal of neurochemistry
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doi：10.1046/j.1471-4159.1994.62062269.x
更新日期：1994-06-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1981.tb04497.x
更新日期：1981-10-01 00:00:00
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pub_type： 杂志文章
doi：10.1111/j.1471-4159.1980.tb07870.x
更新日期：1980-11-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.2008.05730.x
更新日期：2008-12-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章,评审
doi：10.1111/j.1471-4159.2008.05522.x
更新日期：2008-09-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1986.tb12955.x
更新日期：1986-01-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.2004.02752.x
更新日期：2004-11-01 00:00:00
abstract：:A rapid and simple procedure is presented to obtain nearly pure populations of human neuron-like cells from the SH-SY5Y neuroblastoma cell line. Sequential exposure of SH-SY5Y cells to retinoic acid and brain-derived neurotrophic factor in serum-free medium yields homogeneous populations of cells with neuronal morphol...
journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.2000.0750991.x
更新日期：2000-09-01 00:00:00
abstract：:Ischemic stroke is a neurovascular disease treatable by thrombolytic therapy, but the therapy has to be initiated within 3 h of the incident. This therapeutic limitation stems from the secondary injury which results mainly from oxidative stress and inflammation. A potent antioxidant/anti-inflammatory agent, caffeic ac...
journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.2007.04526.x
更新日期：2007-07-01 00:00:00
