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Patient outcomes in KCNQ2 developmental and epileptic encephalopathy

Developmental Medicine &Child Neurology, EarlyView.
Abstract The aim of this study was to review and summarize the literature describing clinically observed or caregiver‐reported and patient‐reported KCNQ2 developmental and epileptic encephalopathy (DEE) outcomes. Three online databases and selected congress proceedings were searched (August 2023).
Grant Maclaine, Michele H. Potashman, Deepshikha Pawar, Sugandh Sharma, Katja Rudell, Jason Lerner, Vlad Coric, Anne T. Berg, John Millichap, Gil L'Italien   +9 more
wiley   +1 more source

An extreme type of new onset refractory status epilepticus with stimulus‐induced seizures in pharmacological isoelectric states

Epilepsia, EarlyView.
Abstract Objective Status epilepticus (SE) is a common neurological emergency associated with high morbidity and mortality. SE is classified as refractory when it persists despite benzodiazepine and second‐line antiseizure medication. Managing refractory SE in the intensive care setting often requires high doses of sedative drugs, which can induce ...
Julie Lévi‐Strauss, Vi‐Huong Nguyen‐Michel, Espártaco Moraes Ribeiro, Virginie Lambrecq, Aurélie Hanin, Sophie Demeret, Vincent Navarro   +6 more
wiley   +1 more source

Voltage-gated potassium channels as therapeutic targets [PDF]

Nature Reviews Drug Discovery, 2009
The human genome encodes 40 voltage-gated K(+) channels (K(V)), which are involved in diverse physiological processes ranging from repolarization of neuronal and cardiac action potentials, to regulating Ca(2+) signalling and cell volume, to driving cellular proliferation and migration. K(V) channels offer tremendous opportunities for the development of
Heike Wulff, Neil A Castle, Luis A Pardo
exaly   +5 more sources

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Voltage-gated potassium channel antibodies

Neurology, 2016
A radioimmunoassay (RIA) to detect antibodies against what was initially believed to be voltage-gated potassium channels (VGKC) in patients with neuromyotonia was established 20 years ago.1 The rationale for this test came from previous RIA assays that detected antibodies against acetylcholine receptors or voltage-gated calcium channels in which the ...
Francesc, Graus, Mark P, Gorman
openaire   +2 more sources

Voltage-gated Potassium Channel Inhibitors

Current Pharmaceutical Design, 1996
Forty years have transpired since tetraethylammonium was first used to selectively inhibit the potassiuin conductance in squid axons. Since then, a large body of work has emerged describing inhibitors of voltage-gated potassium currents in a variety of cells.
W. F. Hopkins, J. L. Miller, G. P. Miljanich   +2 more
openaire   +1 more source

Mechanism of Voltage Gating in Potassium Channels

Science, 2012
Open and Shut Case Voltage-sensing domains (VSDs) control the activity of voltage-gated ion channels to regulate the ion flow that underlies nerve conduction. Structural and biophysical studies have provided insight into voltage gating; however, understanding has been hindered by the lack of a crystal structure of a fully closed ...
Morten Ø, Jensen, Vishwanath, Jogini, David W, Borhani, Abba E, Leffler, Ron O, Dror, David E, Shaw   +5 more
openaire   +2 more sources

Enzymatic activation of voltage-gated potassium channels

Nature, 2006
Voltage-gated ion channels in excitable nerve, muscle, and endocrine cells generate electric signals in the form of action potentials. However, they are also present in non-excitable eukaryotic cells and prokaryotes, which raises the question of whether voltage-gated channels might be activated by means other than changing the voltage difference ...
Yajamana, Ramu, Yanping, Xu, Zhe, Lu
openaire   +2 more sources

Neuronal trafficking of voltage-gated potassium channels

Molecular and Cellular Neuroscience, 2011
The computational ability of CNS neurons depends critically on the specific localization of ion channels in the somatodendritic and axonal membranes. Neuronal dendrites receive synaptic inputs at numerous spines and integrate them in time and space. The integration of synaptic potentials is regulated by voltage-gated potassium (Kv) channels, such as ...
Jensen, Camilla S, Rasmussen, Hanne Borger, Misonou, Hiroaki   +2 more
openaire   +3 more sources

Voltage-Gated Potassium Channels in Cell Proliferation

Physiology, 2004
It is commonly accepted that cells require K+channels to proliferate. The role(s) of K+channels in the process is, however, poorly understood. Cloning of K+channel genes opened the possibility to approach this problem in a way more independent from pharmacological tools.
openaire   +3 more sources

Voltage-gated potassium channels in human ductus arteriosus

The Lancet, 2000
We studied tone in the human ductus arteriosus and show that the constriction to oxygen is due to inhibition of voltage-gated potassium channels and, in the acute phase, is independent of endothelin-1.
E, Michelakis, I, Rebeyka, J, Bateson, P, Olley, L, Puttagunta, S, Archer   +5 more
openaire   +2 more sources