Literature DB >> 11110585

Neurological potassium channelopathies.

M Benatar1.   

Abstract

Potassium channel dysfunction has been implicated in a variety of genetic and acquired neurological disorders that are collectively referred to as the potassium channelopathies. These include acquired neuromyotonia, episodic ataxia type-1, hereditary deafness syndromes, benign familial neonatal convulsions and hypokalaemic periodic paralysis. Insight into potassium channel structure and function is crucial to understanding the pathophysiology of these conditions. This article describes potassium channel structure and function and then outlines what is known about the immunology and genetics of the neurological potassium channelopathies.

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Year:  2000        PMID: 11110585     DOI: 10.1093/qjmed/93.12.787

Source DB:  PubMed          Journal:  QJM        ISSN: 1460-2393


  13 in total

1.  Structural and biophysical properties of a synthetic channel-forming peptide: designing a clinically relevant anion selective pore.

Authors:  U Bukovnik; J Gao; G A Cook; L P Shank; M B Seabra; B D Schultz; T Iwamoto; J Chen; J M Tomich
Journal:  Biochim Biophys Acta       Date:  2011-07-31

2.  A homozygous mutation in KCTD7 links neuronal ceroid lipofuscinosis to the ubiquitin-proteasome system.

Authors:  John F Staropoli; Amel Karaa; Elaine T Lim; Andrew Kirby; Naser Elbalalesy; Stephen G Romansky; Karen B Leydiker; Scott H Coppel; Rosemary Barone; Winnie Xin; Marcy E MacDonald; Jose E Abdenur; Mark J Daly; Katherine B Sims; Susan L Cotman
Journal:  Am J Hum Genet       Date:  2012-06-28       Impact factor: 11.025

Review 3.  Mutational consequences of aberrant ion channels in neurological disorders.

Authors:  Dhiraj Kumar; Rashmi K Ambasta; Pravir Kumar
Journal:  J Membr Biol       Date:  2014-08-14       Impact factor: 1.843

4.  Kv1.1 deletion augments the afferent hypoxic chemosensory pathway and respiration.

Authors:  David D Kline; Maria C F Buniel; Patricia Glazebrook; Ying-Jie Peng; Angelina Ramirez-Navarro; Nanduri R Prabhakar; Diana L Kunze
Journal:  J Neurosci       Date:  2005-03-30       Impact factor: 6.167

5.  Differential expression of voltage-gated K+ currents in medial septum/diagonal band complex neurons exhibiting distinct firing phenotypes.

Authors:  Emilio R Garrido-Sanabria; Miriam G Perez-Cordova; Luis V Colom
Journal:  Neurosci Res       Date:  2011-05-20       Impact factor: 3.304

6.  Progressive myoclonic epilepsy-associated gene KCTD7 is a regulator of potassium conductance in neurons.

Authors:  Régis Azizieh; David Orduz; Patrick Van Bogaert; Tristan Bouschet; Wendy Rodriguez; Serge N Schiffmann; Isabelle Pirson; Marc J Abramowicz
Journal:  Mol Neurobiol       Date:  2011-06-28       Impact factor: 5.682

7.  BK channel properties correlate with neurobehavioral severity in three KCNMA1-linked channelopathy mouse models.

Authors:  Su Mi Park; Cooper E Roache; Philip H Iffland; Hans J Moldenhauer; Katia K Matychak; Amber E Plante; Abby G Lieberman; Peter B Crino; Andrea Meredith
Journal:  Elife       Date:  2022-07-12       Impact factor: 8.713

Review 8.  Physiologic and pathophysiologic consequences of altered sialylation and glycosylation on ion channel function.

Authors:  Deniz Baycin-Hizal; Allan Gottschalk; Elena Jacobson; Sunny Mai; Daniel Wolozny; Hui Zhang; Sharon S Krag; Michael J Betenbaugh
Journal:  Biochem Biophys Res Commun       Date:  2014-06-24       Impact factor: 3.575

Review 9.  Using Xenopus oocytes in neurological disease drug discovery.

Authors:  Steven L Zeng; Leland C Sudlow; Mikhail Y Berezin
Journal:  Expert Opin Drug Discov       Date:  2019-11-01       Impact factor: 6.098

Review 10.  Episodic ataxia type 1: a neuronal potassium channelopathy.

Authors:  Sanjeev Rajakulendran; Stephanie Schorge; Dimitri M Kullmann; Michael G Hanna
Journal:  Neurotherapeutics       Date:  2007-04       Impact factor: 7.620

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