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学科主题临床医学
Erythromelalgia mutation L823R shifts activation and inactivation of threshold sodium channel Nav1.7 to hyperpolarized potentials
Lampert, Angelika1,3,4; Dib-Hajj, Sulayman D.1,3,4; Eastman, Emmanuella M.1,3,4; Tyrrell, Lynda1,3,4; Lin, Zhimiao2; Yang, Yong2; Waxman, Stephen G.1,3,4
关键词Sodium Channel Pain Mutation Patch-clamp Inactivation Erythromelalgia
刊名BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
2009-12-11
DOI10.1016/j.bbrc.2009.09.121
390期:2页:319-324
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Biochemistry & Molecular Biology ; Biophysics
资助者Robert Bosch Foundation ; Fok Ying Tong Educat ion Foundation ; Robert Bosch Foundation ; Fok Ying Tong Educat ion Foundation
研究领域[WOS]Biochemistry & Molecular Biology ; Biophysics
关键词[WOS]SENSOR POSITIVE CHARGES ; OF-FUNCTION MUTATION ; RESPONSIVE ERYTHROMELALGIA ; PRIMARY ERYTHERMALGIA ; STATE INACTIVATION ; NA(V)1.7 CHANNEL ; SKELETAL-MUSCLE ; PAIN DISORDERS ; NEURONS ; ONSET
英文摘要

Erythromelalgia (also termed erythermalgia) is a neuropathic pain syndrome, characterized by severe burning pain combined with redness in the extremities, triggered by mild warmth. The inherited form of erythromelalgia (IEM) has recently been linked to mutations in voltage-gated sodium channel Nav1.7, which is expressed in peripheral nociceptors. Here, we used whole-cell voltage-clamp recordings in HEK293 cells to characterize the IEM mutation L823R, which introduces an additional positive charge into the S4 voltage sensor of domain II. The L823R mutation produces a similar to 15 mV hyperpolarizing shift in the midpoint of activation and also affects the activation slope factor. Closing of the channel from the open state (deactivation) is slowed, increasing the likelihood of the channel remaining in the open state. The L823R mutation induces a similar to 10 mV hyperpolarizing shift in fast-inactivation. L823R is the only naturally-occurring IEM mutation studied thus far to shift fast-inactivation to more negative potentials. We conclude that introduction of an additional charge into the S4 segment of domain 11 of Nav1.7 leads to a pronounced hyperpolarizing shift of activation, a change that is expected to increase nociceptor excitability despite the hyperpolarizing shift in fast-inactivation, which is unique among the IEM mutations. (C) 2009 Elsevier Inc. All rights reserved.

语种英语
所属项目编号NCET-06-0015 ; 111039
资助者Robert Bosch Foundation ; Fok Ying Tong Educat ion Foundation ; Robert Bosch Foundation ; Fok Ying Tong Educat ion Foundation
WOS记录号WOS:000271552400027
Citation statistics
Cited Times:15[WOS]   [WOS Record]     [Related Records in WOS]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/50684
Collection北京大学第一临床医学院_皮肤性病科
作者单位1.Vet Affairs Connecticut Healthcare Syst, Rehabil Res Ctr, West Haven, CT 06516 USA
2.Peking Univ, Hosp 1, Dept Dermatol, Beijing 100034, Peoples R China
3.Yale Univ, Sch Med, Ctr Neurosci & Regenerat Res, New Haven, CT 06520 USA
4.Yale Univ, Sch Med, Dept Neurol, New Haven, CT 06510 USA
Recommended Citation
GB/T 7714
Lampert, Angelika,Dib-Hajj, Sulayman D.,Eastman, Emmanuella M.,et al. Erythromelalgia mutation L823R shifts activation and inactivation of threshold sodium channel Nav1.7 to hyperpolarized potentials[J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,2009,390(2):319-324.
APA Lampert, Angelika.,Dib-Hajj, Sulayman D..,Eastman, Emmanuella M..,Tyrrell, Lynda.,Lin, Zhimiao.,...&Waxman, Stephen G..(2009).Erythromelalgia mutation L823R shifts activation and inactivation of threshold sodium channel Nav1.7 to hyperpolarized potentials.BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,390(2),319-324.
MLA Lampert, Angelika,et al."Erythromelalgia mutation L823R shifts activation and inactivation of threshold sodium channel Nav1.7 to hyperpolarized potentials".BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 390.2(2009):319-324.
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