Abstract：
:Fragile X syndrome (FXS) is a developmental disorder caused by the loss of Fragile X Mental Retardation 1 (FMR1) gene function because of a CGG repeat expansion (> 200 repeats) in the gene. The molecular mechanism(s) linking loss of FMR1 function to the molecular pathology and cognitive/behavioral disability remain unclear. Given the critical role of extracellular signal-regulated kinase (ERK) in synaptic plasticity and neurodevelopment, a number of recent studies have investigated ERK phosphorylation under basal conditions or upon mGluR-induction using neuronal and peripheral tissues from Fmr1 knockout mice and peripheral tissues from FXS patients. However, these reports have presented conflicting results. The current study is the first to focus on the levels of ERK phosphorylation in brain tissue from human FXS patients. In both human brain tissue and brain tissue from Fmr1 knockout mice there was significantly increased phosphorylation of MEK1/2 and ERK. Indeed, treating Fmr1 knockout mice with the MEK1/2 inhibitor SL327 abrogated audiogenic seizure activity, a feature of the Fmr1 knockout mice that replicates the symptom in patients with FXS. These findings suggest that activation of the ERK pathway results in some cardinal cognitive and clinical features in FXS patients and likely have profound translational implications.
journal_name
J Neurochemjournal_title
Journal of neurochemistryauthors
Wang X,Snape M,Klann E,Stone JG,Singh A,Petersen RB,Castellani RJ,Casadesus G,Smith MA,Zhu Xdoi
10.1111/j.1471-4159.2012.07722.xsubject
Has Abstractpub_date
2012-05-01 00:00:00pages
672-9issue
4eissn
0022-3042issn
1471-4159journal_volume
121pub_type
杂志文章abstract：:The Na+ and K+ concentrations in isolated Torpedo marmorata synaptosomes were determined. Synaptosomes made according to the method of Israël et al. have high internal Na+ (290 mM) and low internal K+ (30 mM) concentrations. Modification of the homogenisation media permitted the isolation of synaptosomes which could m...
journal_title：Journal of neurochemistry
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doi：10.1111/j.1471-4159.1981.tb00597.x
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doi：10.1111/j.1471-4159.1983.tb09060.x
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journal_title：Journal of neurochemistry
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doi：10.1111/j.1471-4159.1993.tb13625.x
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1985.tb07107.x
更新日期：1985-01-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1984.tb00920.x
更新日期：1984-08-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.1999.731718.x
更新日期：1999-10-01 00:00:00
abstract：:2,4,5-Trihydroxyphenylalanine (6-OH-DOPA) destroys central and peripheral noradrenergic neurons, while sparing dopaminergic neurons. Previous studies indicate that 6-OH-DOPA toxicity is mediated by the formation of 6-hydroxydopamine. However, levels of 6-hydroxydopamine in brain following peripheral administration of ...
journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1989.tb09194.x
更新日期：1989-05-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1992.tb08914.x
更新日期：1992-07-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.1996.67020473.x
更新日期：1996-08-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.2008.05697.x
更新日期：2008-11-01 00:00:00
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doi：10.1111/j.1471-4159.2005.03363.x
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journal_title：Journal of neurochemistry
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journal_title：Journal of neurochemistry
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journal_title：Journal of neurochemistry
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abstract：:Choline acetyltransferase (ChAT), the enzyme synthesizing acetylcholine, is known to be activated by brain derived neurotrophic factor (BDNF). We found that the specific removal of the carbohydrate polysialic acid (PSA) significantly increased BDNF-induced ChAT-activity in embryonic septal neurons. Using a p75 neurotr...
journal_title：Journal of neurochemistry
pub_type： 杂志文章
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abstract：:We have investigated the isozymes of a phosphoinositide-specific phospholipase C (PLC) in bovine retina using several monoclonal antisera to PLCbeta1, gamma1, and delta1. Immunoblot analysis showed that all three isozymes were present in the retina. Immunocytochemical localization in frozen bovine retina sections show...
journal_title：Journal of neurochemistry
pub_type： 杂志文章
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更新日期：1998-01-01 00:00:00
