Treatment of myotonia congenita with retigabine in mice.
Action Potentials
/ drug effects
Animals
Behavior, Animal
/ drug effects
Carbamates
/ therapeutic use
Chloride Channels
/ genetics
In Vitro Techniques
KCNQ Potassium Channels
/ drug effects
Membrane Potentials
/ drug effects
Membrane Transport Modulators
/ therapeutic use
Mice
Muscle Contraction
/ drug effects
Muscle, Skeletal
/ drug effects
Myotonia Congenita
/ drug therapy
Phenylenediamines
/ therapeutic use
Psychomotor Performance
/ drug effects
Action potential
Excitability
Kv7
Muscle
Muscle contraction
Myotonia
Persistent inward current
Potassium channel
Retigabine
Sodium channel
Journal
Experimental neurology
ISSN: 1090-2430
Titre abrégé: Exp Neurol
Pays: United States
ID NLM: 0370712
Informations de publication
Date de publication:
05 2019
05 2019
Historique:
received:
25
09
2018
revised:
10
01
2019
accepted:
06
02
2019
pubmed:
11
2
2019
medline:
25
2
2020
entrez:
11
2
2019
Statut:
ppublish
Résumé
Patients with myotonia congenita suffer from muscle stiffness caused by muscle hyperexcitability. Although loss-of-function mutations in the ClC-1 muscle chloride channel have been known for 25 years to cause myotonia congenita, this discovery has led to little progress on development of therapy. Currently, treatment is primarily focused on reducing hyperexcitability by blocking Na
Identifiants
pubmed: 30738808
pii: S0014-4886(18)30508-9
doi: 10.1016/j.expneurol.2019.02.002
pmc: PMC6431423
mid: NIHMS1521348
pii:
doi:
Substances chimiques
CLC-1 channel
0
Carbamates
0
Chloride Channels
0
KCNQ Potassium Channels
0
Membrane Transport Modulators
0
Phenylenediamines
0
ezogabine
12G01I6BBU
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
52-59Subventions
Organisme : NINDS NIH HHS
ID : R01 NS082354
Pays : United States
Organisme : NINDS NIH HHS
ID : R15 NS099850
Pays : United States
Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.
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