Sodium channel SCN1A and epilepsy: Mutations and mechanisms

A Escayg, AL Goldin - Epilepsia, 2010 - Wiley Online Library
Mutations in a number of genes encoding voltage‐gated sodium channels cause a variety of
epilepsy syndromes in humans, including genetic (generalized) epilepsy with febrile …

Sodium channel mutations in epilepsy and other neurological disorders

MH Meisler, JA Kearney - The Journal of clinical …, 2005 - Am Soc Clin Investig
Since the first mutations of the neuronal sodium channel SCN1A were identified 5 years
ago, more than 150 mutations have been described in patients with epilepsy. Many are …

SCN1A mutations and epilepsy

JC Mulley, IE Scheffer, S Petrou, LM Dibbens… - Human …, 2005 - Wiley Online Library
SCN1A is part of the SCN1A‐SCN2A‐SCN3A gene cluster on chromosome 2q24 that
encodes for α pore forming subunits of sodium channels. The 26 exons of SCN1A are …

Sodium channel inactivation: molecular determinants and modulation

W Ulbricht - Physiological reviews, 2005 - journals.physiology.org
Voltage-gated sodium channels open (activate) when the membrane is depolarized and
close on repolarization (deactivate) but also on continuing depolarization by a process …

Molecular basis of an inherited epilepsy

C Lossin, DW Wang, TH Rhodes, CG Vanoye… - Neuron, 2002 - cell.com
Epilepsy is a common neurological condition that reflects neuronal hyperexcitability arising
from largely unknown cellular and molecular mechanisms. In generalized epilepsy with …

Mechanisms of sodium channel inactivation

AL Goldin - Current opinion in neurobiology, 2003 - Elsevier
Rapid inactivation of sodium channels is crucial for the normal electrical activity of excitable
cells. There are many different types of inactivation, including fast, slow and ultra-slow, and …

Altered function of the SCN1A voltage-gated sodium channel leads to γ-aminobutyric acid-ergic (GABAergic) interneuron abnormalities

MS Martin, K Dutt, LA Papale, CM Dubé… - Journal of Biological …, 2010 - ASBMB
Voltage-gated sodium channels are required for the initiation and propagation of action
potentials. Mutations in the neuronal voltage-gated sodium channel SCN1A are associated …

De-novo mutations of the sodium channel gene SCN1A in alleged vaccine encephalopathy: a retrospective study

SF Berkovic, L Harkin, JM McMahon… - The Lancet …, 2006 - thelancet.com
Background Vaccination, particularly for pertussis, has been implicated as a direct cause of
an encephalopathy with refractory seizures and intellectual impairment. We postulated that …

Aberrant epilepsy-associated mutant Nav1.6 sodium channel activity can be targeted with cannabidiol

RR Patel, C Barbosa, T Brustovetsky, N Brustovetsky… - Brain, 2016 - academic.oup.com
Mutations in brain isoforms of voltage-gated sodium channels have been identified in
patients with distinct epileptic phenotypes. Clinically, these patients often do not respond …

Fever, genes, and epilepsy

S Baulac, I Gourfinkel-An, R Nabbout… - The lancet …, 2004 - thelancet.com
About 13% of patients with epilepsy have a history of febrile seizures (FS). Studies of familial
forms suggest a genetic component to the epidemiological link. Indeed, in certain …