Published in Epilepsy Curr on January 01, 2010
Mutation of ARX causes abnormal development of forebrain and testes in mice and X-linked lissencephaly with abnormal genitalia in humans. Nat Genet (2002) 4.01
De novo mutations in the gene encoding STXBP1 (MUNC18-1) cause early infantile epileptic encephalopathy. Nat Genet (2008) 2.98
ARX, a novel Prd-class-homeobox gene highly expressed in the telencephalon, is mutated in X-linked mental retardation. Hum Mol Genet (2002) 2.30
Munc18-1 in secretion: lonely Munc joins SNARE team and takes control. Trends Neurosci (2007) 1.96
Expansion of the first PolyA tract of ARX causes infantile spasms and status dystonicus. Neurology (2007) 1.90
Identification of Arx transcriptional targets in the developing basal forebrain. Hum Mol Genet (2008) 1.58
Infantile spasms, dystonia, and other X-linked phenotypes caused by mutations in Aristaless related homeobox gene, ARX. Brain Dev (2002) 1.56
Targeted loss of Arx results in a developmental epilepsy mouse model and recapitulates the human phenotype in heterozygous females. Brain (2009) 1.52
A triplet repeat expansion genetic mouse model of infantile spasms syndrome, Arx(GCG)10+7, with interneuronopathy, spasms in infancy, persistent seizures, and adult cognitive and behavioral impairment. J Neurosci (2009) 1.48
ARX: a gene for all seasons. Curr Opin Genet Dev (2006) 1.46
A longer polyalanine expansion mutation in the ARX gene causes early infantile epileptic encephalopathy with suppression-burst pattern (Ohtahara syndrome). Am J Hum Genet (2007) 1.36
X-linked lissencephaly with abnormal genitalia as a tangential migration disorder causing intractable epilepsy: proposal for a new term, "interneuronopathy". J Child Neurol (2005) 1.25
Infantile spasms is associated with deletion of the MAGI2 gene on chromosome 7q11.23-q21.11. Am J Hum Genet (2008) 1.19
Neuroanatomical distribution of ARX in brain and its localisation in GABAergic neurons. Brain Res Mol Brain Res (2004) 1.19
Models of pediatric epilepsies: strategies and opportunities. Epilepsia (2006) 1.12
Aristaless-related homeobox gene, the gene responsible for West syndrome and related disorders, is a Groucho/transducin-like enhancer of split dependent transcriptional repressor. Neuroscience (2007) 1.11
Infantile spasms. Pediatr Neurol (2001) 1.07
FACS-array gene expression analysis during early development of mouse telencephalic interneurons. Dev Neurobiol (2008) 1.05
Aristaless-related homeobox gene disruption leads to abnormal distribution of GABAergic interneurons in human neocortex: evidence based on a case of X-linked lissencephaly with abnormal genitalia (XLAG). Acta Neuropathol (2008) 0.94
IGF-1 receptor-mediated ERK/MAPK signaling couples status epilepticus to progenitor cell proliferation in the subgranular layer of the dentate gyrus. Glia (2008) 1.21
Differentiation and functional incorporation of embryonic stem cell-derived GABAergic interneurons in the dentate gyrus of mice with temporal lobe epilepsy. J Neurosci (2012) 1.13
Region-specific differentiation of embryonic stem cell-derived neural progenitor transplants into the adult mouse hippocampus following seizures. J Neurosci Res (2008) 1.07
Derivation and isolation of NKX2.1-positive basal forebrain progenitors from human embryonic stem cells. Stem Cells Dev (2013) 1.07
Status epilepticus-induced somatostatinergic hilar interneuron degeneration is regulated by striatal enriched protein tyrosine phosphatase. J Neurosci (2007) 1.07
Teratocarcinoma formation in embryonic stem cell-derived neural progenitor hippocampal transplants. Cell Transplant (2012) 0.93
CXCL12-mediated guidance of migrating embryonic stem cell-derived neural progenitors transplanted into the hippocampus. PLoS One (2010) 0.92
Postnatal cellular contributions of the hippocampus subventricular zone to the dentate gyrus, corpus callosum, fimbria, and cerebral cortex. J Comp Neurol (2006) 0.91
Oxidative damage and defective DNA repair is linked to apoptosis of migrating neurons and progenitors during cerebral cortex development in Ku70-deficient mice. Cereb Cortex (2004) 0.91
Embryonic stem cell-derived neural precursor grafts for treatment of temporal lobe epilepsy. Neurotherapeutics (2009) 0.86
PARP cleavage, DNA fragmentation, and pyknosis during excitotoxin-induced neuronal death. Exp Neurol (2003) 0.85
DNA damage and nonhomologous end joining in excitotoxicity: neuroprotective role of DNA-PKcs in kainic acid-induced seizures. Hippocampus (2005) 0.84
The DNA-PK catalytic subunit regulates Bax-mediated excitotoxic cell death by Ku70 phosphorylation. Brain Res (2009) 0.83
The Art of Setting Limits: Lessons from GABAergic Interneuron Transplantation. Epilepsy Curr (2015) 0.75
CLC-3 Takes a Quantum Leap Toward Understanding Mechanisms of GABA Release. Epilepsy Curr (2012) 0.75
Trekking through the telencephalon: hepatocyte growth factor-mediated guidance for parvalbumin-expressing interneurons. Epilepsy Curr (2010) 0.75
Tangled roots: digging deeper into astrocyte or interneuron dysfunction in temporal lobe epilepsy. Epilepsy Curr (2011) 0.75
SAVAs: Molecular Snipers for Silencing GABAergic Interneurons. Epilepsy Curr (2012) 0.75
Blocking Astrocyte Transformation at the Dysfunctional Blood Brain Barrier. Epilepsy Curr (2015) 0.75
Should I stay or should I go? Epilepsy Curr (2011) 0.75
Synaptic Sweet Talk: How Do Perineuronal Nets Contribute to Epileptogenesis and Neuroplasticity? Epilepsy Curr (2016) 0.75
Immature Interneurons Create a Lasting Impression. Epilepsy Curr (2015) 0.75