Developmental and epileptic encephalopathies: from genetic heterogeneity to phenotypic continuum

R Guerrini, V Conti, M Mantegazza… - Physiological …, 2023‏ - journals.physiology.org
Developmental and epileptic encephalopathies (DEEs) are a heterogeneous group of
disorders characterized by early-onset, often severe epileptic seizures and EEG …

The human phenotype ontology in 2017

S Köhler, NA Vasilevsky, M Engelstad… - Nucleic acids …, 2017‏ - academic.oup.com
Deep phenoty** has been defined as the precise and comprehensive analysis of
phenotypic abnormalities in which the individual components of the phenotype are observed …

Genetic studies in intellectual disability and related disorders

LELM Vissers, C Gilissen, JA Veltman - Nature Reviews Genetics, 2016‏ - nature.com
Genetic factors play a major part in intellectual disability (ID), but genetic studies have been
complicated for a long time by the extreme clinical and genetic heterogeneity. Recently …

Ion channels in genetic epilepsy: from genes and mechanisms to disease-targeted therapies

J Oyrer, S Maljevic, IE Scheffer, SF Berkovic… - Pharmacological …, 2018‏ - Elsevier
Epilepsy is a common and serious neurologic disease with a strong genetic component.
Genetic studies have identified an increasing collection of disease-causing genes. The …

Structures of the human HCN1 hyperpolarization-activated channel

CH Lee, R MacKinnon - Cell, 2017‏ - cell.com
Hyperpolarization-activated cyclic nucleotide-gated (HCN) channels underlie the control of
rhythmic activity in cardiac and neuronal pacemaker cells. In HCN, the polarity of voltage …

[PDF][PDF] The human phenotype ontology in 2017

S Kohler, NA Vasilevsky, M Engelstad, E Foster… - 2017‏ - repository.ubn.ru.nl
Deep phenoty** has been defined as the precise and comprehensive analysis of
phenotypic abnormalities in which the individual components of the phenotype are observed …

Phenotypic landscape of schizophrenia-associated genes defines candidates and their shared functions

SB Thyme, LM Pieper, EH Li, S Pandey, Y Wang… - Cell, 2019‏ - cell.com
Genomic studies have identified hundreds of candidate genes near loci associated with risk
for schizophrenia. To define candidates and their functions, we mutated zebrafish orthologs …

Advancing epilepsy genetics in the genomic era

CT Myers, HC Mefford - Genome medicine, 2015‏ - Springer
Epilepsy is a group of disorders characterized by recurrent seizures, and is one of the most
common neurological conditions. The genetic basis of epilepsy is clear from epidemiological …

Improving diagnosis and broadening the phenotypes in early-onset seizure and severe developmental delay disorders through gene panel analysis

N Trump, A McTague, H Brittain… - Journal of medical …, 2016‏ - jmg.bmj.com
Background We sought to investigate the diagnostic yield and mutation spectrum in
previously reported genes for early-onset epilepsy and disorders of severe developmental …

De novo loss- or gain-of-function mutations in KCNA2 cause epileptic encephalopathy

S Syrbe, UBS Hedrich, E Riesch, T Djémié, S Müller… - Nature …, 2015‏ - nature.com
Epileptic encephalopathies are a phenotypically and genetically heterogeneous group of
severe epilepsies accompanied by intellectual disability and other neurodevelopmental …