Abstract：
:Globoid cell leukodystrophy (GLD, Krabbe Disease) is a lysosomal storage disease, resulting from the genetic deficiency of galactosylceramidase (GALC). This disease is marked by accumulation of the cytotoxic lipid psychosine (Psy). Psychosine is known to induce oxidative stress in cultured cells, and this stress can be ameliorated through co-treatment with the antioxidant N-acetyl cysteine (NAC). Oxidative stress has also been observed in vivo in the mouse model of GLD, the Twitcher mouse (Twi). We hypothesized that treating oxidative stress with NAC; either alone or in combination with bone marrow transplant (BMT) would improve the course of disease. All breeding cages were maintained on water containing NAC. Once born, the pups received IP boluses of NAC three times per week, and were maintained on NAC-containing water. A separate cohort of animals received the same regimen of NAC in addition to a BMT on post-natal days 2-3. Although NAC lowers the level of oxidized proteins in the brains of Twi mice, and dramatically improves immunohistochemical markers of disease, neither treatment results in any clinical improvements in the Twi mouse. Our data suggest that oxidative stress may be sufficiently down-stream in the pathogenic cascade initiated by Psy accumulation as to be difficult or impossible to treat with standard pharmacologic agents. It is possible that NAC may synergize with other therapies or combinations of therapies. A better understanding of the initiating effects of Psy toxicity and oxidative damage may uncover treatable therapeutic targets.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Hawkins-Salsbury JA,Qin EY,Reddy AS,Vogler CA,Sands MSdoi
10.1016/j.expneurol.2012.07.013subject
Has Abstractpub_date
2012-10-01 00:00:00pages
444-52issue
2eissn
0014-4886issn
1090-2430pii
S0014-4886(12)00290-7journal_volume
237pub_type
杂志文章abstract：:Electrophysiological studies on preganglionic neurons (PGNs) in the dorsal motor nucleus (DMN) of the vagus nerve has been hampered by technical limitations. Conventional electrical stimulation of the vagus nerve with cathodal square-wave pulses activates both preganglionic and afferent fibers. Thus, some PGNs cannot ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90076-2
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abstract：:Therapeutic hypothermia is a potential protective strategy after stroke. The present study evaluated the neurovascular protective potential of pharmacological hypothermia induced by the neurotensin receptor 1 agonist HPI-201 after severe ischemic stroke. Adult C57BL/6 mice were subjected to filament insertion-induced ...
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journal_title：Experimental neurology
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6944
更新日期：1998-12-01 00:00:00
abstract：:The increasing demand on diagnostic assays that are sensitive and specific for pathogenic antibodies, and the interest in identifying new antigens, prompted the development of cell-based assays for the detection of autoantibodies in myasthenia gravis and other autoimmune disorders. Cell-based assays were initially use...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2015.01.011
更新日期：2015-08-01 00:00:00
abstract：:Ischemic stroke is a devastating brain injury and an important cause of neurologic disability worldwide and across the lifespan. Despite the physical, social, and economic burdens of this disease there is only a single approved medicine for the treatment of acute stroke, and its use is unfortunately limited to the sma...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90161-5
更新日期：1986-07-01 00:00:00
abstract：:The tooth pulp-evoked jaw-opening reflex was studied in the barbiturate-anesthetized cat. At liminal intensity of the stimulus, a stable short-latency response was obtained in the digastricus and in the tongue. At a higher stimulus intensity, there occasionally appeared to be a prolonged discharge of variable duration...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90135-4
更新日期：1986-04-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2013.04.003
更新日期：2013-09-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
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journal_title：Experimental neurology
pub_type： 评论,杂志文章,评审
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更新日期：2007-02-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1997.6427
更新日期：1997-04-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.2000.7379
更新日期：2000-06-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/s0014-4886(03)00054-2
更新日期：2003-06-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2014.02.004
更新日期：2014-09-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(87)90179-8
更新日期：1987-04-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2015.05.018
更新日期：2015-12-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2014.12.005
更新日期：2015-02-01 00:00:00
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journal_title：Experimental neurology
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doi：10.1016/s0014-4886(03)00354-6
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journal_title：Experimental neurology
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journal_title：Experimental neurology
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doi：10.1016/j.expneurol.2016.01.017
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(86)90077-4
更新日期：1986-02-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
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更新日期：1988-02-01 00:00:00
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journal_title：Experimental neurology
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7272
更新日期：2000-04-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2005.08.028
更新日期：2006-01-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1996.6365
更新日期：1997-04-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.02.015
更新日期：2012-05-01 00:00:00
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journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2007.12.004
更新日期：2008-04-01 00:00:00
abstract：BACKGROUND:Novel strategies to improve hand function after stroke are needed. Electromyography-triggered functional neuromuscular stimulation (EMG-FNMS) and repetitive transcranial magnetic stimulation (rTMS) are promising techniques to facilitate recovery of sensory-motor hand dysfunction after stroke. OBJECTIVE:To i...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2011.04.010
更新日期：2011-07-01 00:00:00
