Literature DB >> 25037091

Muscle channelopathies.

Jeffrey Statland1, Lauren Phillips2, Jaya R Trivedi3.   

Abstract

Skeletal muscle channelopathies are rare heterogeneous diseases with marked genotypic and phenotypic variability. Despite advances in understanding of the molecular pathology of these disorders, the diverse phenotypic manifestations remain a challenge in diagnosis and therapeutics. These disorders can cause lifetime disability and affect quality of life. There is no treatment of these disorders approved by the US Food and Drug Administration at this time. Recognition and treatment of symptoms might reduce morbidity and improve quality of life. This article summarizes the clinical manifestations, diagnostic studies, pathophysiology, and treatment options in nondystrophic myotonia, congenital myasthenic syndrome, and periodic paralyses.
Copyright © 2014 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Channelopathies; Congenital myasthenic syndrome; Ion channel; Nondystrophic myotonia; Periodic paralysis

Mesh:

Substances:

Year:  2014        PMID: 25037091     DOI: 10.1016/j.ncl.2014.04.002

Source DB:  PubMed          Journal:  Neurol Clin        ISSN: 0733-8619            Impact factor:   3.806


  4 in total

Review 1.  Guidelines on clinical presentation and management of nondystrophic myotonias.

Authors:  Bas C Stunnenberg; Samantha LoRusso; W David Arnold; Richard J Barohn; Stephen C Cannon; Bertrand Fontaine; Robert C Griggs; Michael G Hanna; Emma Matthews; Giovanni Meola; Valeria A Sansone; Jaya R Trivedi; Baziel G M van Engelen; Savine Vicart; Jeffrey M Statland
Journal:  Muscle Nerve       Date:  2020-05-27       Impact factor: 3.217

Review 2.  Skeletal Muscle Channelopathies.

Authors:  Lauren Phillips; Jaya R Trivedi
Journal:  Neurotherapeutics       Date:  2018-10       Impact factor: 7.620

3.  A Restricted Repertoire of De Novo Mutations in ITPR1 Cause Gillespie Syndrome with Evidence for Dominant-Negative Effect.

Authors:  Meriel McEntagart; Kathleen A Williamson; Jacqueline K Rainger; Ann Wheeler; Anne Seawright; Elfride De Baere; Hannah Verdin; L Therese Bergendahl; Alan Quigley; Joe Rainger; Abhijit Dixit; Ajoy Sarkar; Eduardo López Laso; Rocio Sanchez-Carpintero; Jesus Barrio; Pierre Bitoun; Trine Prescott; Ruth Riise; Shane McKee; Jackie Cook; Lisa McKie; Berten Ceulemans; Françoise Meire; I Karen Temple; Fabienne Prieur; Jonathan Williams; Penny Clouston; Andrea H Németh; Siddharth Banka; Hemant Bengani; Mark Handley; Elisabeth Freyer; Allyson Ross; Veronica van Heyningen; Joseph A Marsh; Frances Elmslie; David R FitzPatrick
Journal:  Am J Hum Genet       Date:  2016-04-21       Impact factor: 11.025

4.  Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity.

Authors:  Michela De Bellis; Roberta Carbonara; Julien Roussel; Alessandro Farinato; Ada Massari; Sabata Pierno; Marilena Muraglia; Filomena Corbo; Carlo Franchini; Maria Rosaria Carratù; Annamaria De Luca; Diana Conte Camerino; Jean-François Desaphy
Journal:  Neuropharmacology       Date:  2016-10-13       Impact factor: 5.250

  4 in total

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