STRADalpha deficiency results in aberrant mTORC1 signaling during corticogenesis in humans and mice.

No abstract is available for this paper.

• Publication year

  2010

• Venue

  Journal of Clinical Investigation

• Publication date

  2010-05-03

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1172/JCI41592PMID 20424326PMCID PMC2860905
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• mTOR and HIF-1α-mediated tumor metabolism in an LKB1 mouse model of Peutz-Jeghers syndrome

  2009cited by this paper
• ATP and MO25α Regulate the Conformational State of the STRADα Pseudokinase and Activation of the LKB1 Tumour Suppressor

  2009cited by this paper
• Loss of Tsc2 in radial glia models the brain pathology of tuberous sclerosis complex in the mouse

  2009cited by this paper
• Structure of the LKB1-STRAD-MO25 Complex Reveals an Allosteric Mechanism of Kinase Activation

  2009cited by this paper
• The Mammalian Target of Rapamycin Signaling Pathway Mediates Epileptogenesis in a Model of Temporal Lobe Epilepsy

  2009cited by this paper
• Reversal of learning deficits in a Tsc2+/− mouse model of tuberous sclerosis

  2008cited by this paper
• Rapamycin treatment for a child with germline PTEN mutation

  2008cited by this paper
• Magnetic resonance imaging detects differences in migration between primary and immortalized neural stem cells.

  2008cited by this paper
• STRADalpha regulates LKB1 localization by blocking access to importin-alpha, and by association with Crm1 and exportin-7.

  2008cited by this paper
• Polyhydramnios, megalencephaly and symptomatic epilepsy caused by a homozygous 7-kilobase deletion in LYK5.

  2007cited by this paper
• Divergent roles of ApoER2 and Vldlr in the migration of cortical neurons

  2007cited by this paper
• LKB1/STRAD promotes axon initiation during neuronal polarization.

  2007cited by this paper
• RAS/ERK Signaling Promotes Site-specific Ribosomal Protein S6 Phosphorylation via RSK and Stimulates Cap-dependent Translation*

  2007cited by this paper
• A Mouse Model of Tuberous Sclerosis: Neuronal Loss of Tsc1 Causes Dysplastic and Ectopic Neurons, Reduced Myelination, Seizure Activity, and Limited Survival

  2007cited by this paper
• LKB1 Regulates Neuronal Migration and Neuronal Differentiation in the Developing Neocortex through Centrosomal Positioning

  2007cited by this paper
• Neuronal subtype specification in the cerebral cortex

  2007cited by this paper
• mTOR pathway as a target in tissue hypertrophy.

  2007cited by this paper
• LKB1 and SAD kinases define a pathway required for the polarization of cortical neurons.

  2007cited by this paper
• Structure-Specific Patterns of Neural Stem Cell Engraftment After Transplantation in the Adult Mouse Brain

  2006cited by this paper
• Regulation of the energy sensor AMP-activated protein kinase by antigen receptor and Ca2+ in T lymphocytes

  2006cited by this paper
• Pten regulates neuronal arborization and social interaction in mice.

  2006cited by this paper
• The tuberous sclerosis complex.

  2006cited by this paper
• In vivo electroporation in the embryonic mouse central nervous system

  2006cited by this paper
• [p27Kip1 independently promotes neuronal differentiation and migration in the cerebral cortex].

  2006cited by this paper
• TOR signaling in growth and metabolism.

  2006cited by this paper
• Rapamycin causes regression of astrocytomas in tuberous sclerosis complex

  2006cited by this paper
• Recessive symptomatic focal epilepsy and mutant contactin-associated protein-like 2.

  2006cited by this paper
• p27kip1 independently promotes neuronal differentiation and migration in the cerebral cortex.

  2006cited by this paper
• Mammalian SAD Kinases Are Required for Neuronal Polarization

  2005cited by this paper
• Subset of individuals with autism spectrum disorders and extreme macrocephaly associated with germline PTEN tumour suppressor gene mutations

  2005cited by this paper
• Regulation of neuronal morphology and function by the tumor suppressors Tsc1 and Tsc2

  2005cited by this paper
• The LKB1 tumor suppressor negatively regulates mTOR signaling.

  2004cited by this paper
• Inaugural Article: The tumor suppressor LKB1 kinase directly activates AMP-activated kinase and regulates apoptosis in response to energy stress

  2004cited by this paper
• mTOR cascade activation distinguishes tubers from focal cortical dysplasia

  2004cited by this paper
• Complete polarization of single intestinal epithelial cells upon activation of LKB1 by STRAD.

  2004cited by this paper
• LKB1 is a master kinase that activates 13 kinases of the AMPK subfamily, including MARK/PAR‐1

  2004cited by this paper
• Regulation of the TSC pathway by LKB1: evidence of a molecular link between tuberous sclerosis complex and Peutz-Jeghers syndrome.

  2004cited by this paper
• 5-Aminoimidazole-4-carboxamide riboside induces apoptosis in Jurkat cells, but the AMP-activated protein kinase is not involved.

  2003cited by this paper
• Complexes between the LKB1 tumor suppressor, STRADα/β and MO25α/β are upstream kinases in the AMP-activated protein kinase cascade

  2003cited by this paper
• MO25α/β interact with STRADα/β enhancing their ability to bind, activate and localize LKB1 in the cytoplasm

  2003cited by this paper
• Management of cellular energy by the AMP‐activated protein kinase system

  2003cited by this paper
• TSC2 mediates cellular energy response to control cell growth and survival.

  2003cited by this paper
• Rheb GTPase is a direct target of TSC2 GAP activity and regulates mTOR signaling.

  2003cited by this paper
• Activation of the tumour suppressor kinase LKB1 by the STE20‐like pseudokinase STRAD

  2003cited by this paper
• MO25alpha/beta interact with STRADalpha/beta enhancing their ability to bind, activate and localize LKB1 in the cytoplasm.

  2003cited by this paper
• Protean PTEN: form and function.

  2002cited by this paper
• Role of Lkb1, the causative gene of Peutz–Jegher's syndrome, in embryogenesis and polyposis

  2002cited by this paper
• Loss of the Lkb1 tumour suppressor provokes intestinal polyposis but resistance to transformation

  2002cited by this paper
• Growth arrest by the LKB1 tumor suppressor: induction of p21(WAF1/CIP1).

  2002cited by this paper
• Growth arrest by the LKB 1 tumor suppressor : induction of p 21 WAF 1 / CIP 1

  2002cited by this paper
• Vascular Abnormalities and Deregulation of VEGF in Lkb1-Deficient Mice

  2001cited by this paper
• Loss of cytoplasmic retention ability of mutant LKB1 found in Peutz-Jeghers syndrome patients.

  1999cited by this paper
• The Tumor Suppressor, PTEN/MMAC1, Dephosphorylates the Lipid Second Messenger, Phosphatidylinositol 3,4,5-Trisphosphate*

  1998cited by this paper
• Translational induction of the c-myc oncogene via activation of the FRAP/TOR signalling pathway

  1998cited by this paper
• Peutz-Jeghers syndrome is caused by mutations in a novel serine threonine kinase.

  1998cited by this paper

• Delayed forebrain excitatory and inhibitory neurogenesis in STRADA-related megalencephaly via mTOR hyperactivity.

  2026cites this paper
• The Clinical Spectrum and Neurodevelopmental Pathogenesis of KPTN-Related Disorder in a Mouse Model.

  2026cites this paper
• Delayed forebrain excitatory and inhibitory neurogenesis in STRADA-related megalencephaly via mTOR hyperactivity

  2025cites this paper
• An overview of the value of mTOR inhibitors to the treatment of epilepsy: the evidence to date

  2025cites this paper
• mTORopathies in Epilepsy and Neurodevelopmental Disorders: The Future of Therapeutics and the Role of Gene Editing

  2025cites this paper
• Functions of LKB1 in neural crest development: The story unfolds

  2023cites this paper
• The Evolving Landscape of Therapeutics for Epilepsy in Tuberous Sclerosis Complex

  2023cites this paper
• Altered Extracellular Matrix as an Alternative Risk Factor for Epileptogenicity in Brain Tumors

  2022cites this paper
• The organization and function of the Golgi apparatus in dendrite development and neurological disorders

  2022cites this paper
• Mechanistic target of rapamycin signaling in human nervous system development and disease

  2022cites this paper
• Convergent and Divergent Mechanisms of Epileptogenesis in mTORopathies

  2021cites this paper
• STRADA‐mutant human cortical organoids model megalencephaly and exhibit delayed neuronal differentiation

  2021cites this paper
• Homozygous missense STRADA mutation in a patient with polyhydramnios, megalencephaly and symptomatic epilepsy syndrome.

  2021cites this paper
• The Putative Role of mTOR Inhibitors in Non-tuberous Sclerosis Complex-Related Epilepsy

  2021cites this paper
• Epilepsy in the mTORopathies: opportunities for precision medicine

  2021cites this paper
• Precision Therapy for Epilepsy Related to Brain Malformations

  2021cites this paper
• Corrigendum: Convergent and Divergent Mechanisms of Epileptogenesis in mTORopathies

  2021cites this paper
• Current Approaches and Future Directions for the Treatment of mTORopathies

  2021cites this paper
• Across-breed validation study confirms and identifies new loci associated with sexual precocity in Brahman and Nellore cattle.

  2020cites this paper
• Microglial mTOR is Neuronal Protective and Antiepileptogenic in the Pilocarpine Model of Temporal Lobe Epilepsy

  2020influential citation
• Multimodal Analysis of STRADA Function in Brain Development

  2020cites this paper
• Sevoflurane Postconditioning Reduces Hypoxia-Reoxygenation Injury in H9C2 Embryonic Rat Cardiomyocytes and Targets the STRADA Gene by Upregulating microRNA-107

  2020cites this paper
• Anticonvulsant mechanisms of the ketogenic diet and caloric restriction.

  2020cites this paper
• Mechanisms of axon polarization in pyramidal neurons.

  2020cites this paper
• Therapeutic role of targeting mTOR signaling and neuroinflammation in epilepsy.

  2020cites this paper
• MIR3607 regulates cerebral cortex development via activation of Wnt/βCat signaling

  2019cites this paper
• New insights into a spectrum of developmental malformations related to mTOR dysregulations: challenges and perspectives

  2019cites this paper
• [Correlation of phosphorylated S6 protein expression in blood and brain tissue in mice and rats with kainic acid-induced seizure].

  2019cites this paper
• GATORopathies: The role of amino acid regulatory gene mutations in epilepsy and cortical malformations

  2019cites this paper
• Golgi Complex Dynamics and Its Implication in Prevalent Neurological Disorders

  2019cites this paper
• Mechanistic Target of Rapamycin Pathway in Epileptic Disorders

  2019cites this paper
• mTOR dysregulation and tuberous sclerosis-related epilepsy

  2018cites this paper
• Novel Homozygous Deletion in STRADA Gene Associated With Polyhydramnios, Megalencephaly, and Epilepsy in 2 Siblings: Implications for Diagnosis and Treatment

  2018cites this paper
• The mTOR pathway in treatment of epilepsy: a clinical update.

  2018cites this paper
• SUMOylation regulates LKB1 localization and its oncogenic activity in liver cancer

  2018cites this paper
• Disorders of neurogenesis and cortical development

  2018cites this paper
• Targeting the Mammalian Target of Rapamycin for Epileptic Encephalopathies and Malformations of Cortical Development

  2018cites this paper
• Versatile Roles of LKB1 Kinase Signaling in Neural Development and Homeostasis

  2018cites this paper
• DEPDC5 and NPRL3 modulate cell size, filopodial outgrowth, and localization of mTOR in neural progenitor cells and neurons.

  2018cites this paper
• Asymmetric Golgi Repositioning: A Prerequisite for Appropriate Dendrite Formation in Adult-Born Neurons

  2018cites this paper
• Inflammation in Epileptic Encephalopathies.

  2017cites this paper
• Micellar nanocomplexes for biomagnetic delivery of intracellular proteins to dictate axon formation during neuronal development.

  2017cites this paper
• Using Case Studies to Promote Student Engagement in Primary Literature Data Analysis and Evaluation.

  2017influential citation
• Tuberous Sclerosis and Other mTORopathies

  2017cites this paper
• Repositioning of Somatic Golgi Apparatus Is Essential for the Dendritic Establishment of Adult-Born Hippocampal Neurons

  2017cites this paper
• Neurobehavioral comorbidities of epilepsy: Role of inflammation

  2017cites this paper
• Neuroinflammation in epileptogenesis: Insights and translational perspectives from new models of epilepsy

  2017cites this paper
• Editorial: In vivo Cell Biology of Cerebral Cortical Development and Its Related Neurological Disorders

  2016cites this paper
• Normalizing translation through 4E-BP prevents mTOR-driven cortical mislamination and ameliorates aberrant neuron integration

  2016cites this paper
• The mTOR signalling cascade: paving new roads to cure neurological disease

  2016cites this paper
• mTOR Inhibitors in Children: Current Indications and Future Directions in Neurology

  2016cites this paper
• Sending Mixed Signals: The Expanding Role of Molecular Cascade Mutations in Malformations of Cortical Development and Epilepsy

  2016cites this paper
• Convulsive seizures from experimental focal cortical dysplasia occur independently of cell misplacement

  2016cites this paper
• Corrigendum: Molecular Pathways Underlying Projection Neuron Production and Migration during Cerebral Cortical Development

  2016influential citation
• Genetic animal models of malformations of cortical development and epilepsy.

  2016cites this paper
• mTOR Inhibition in Epilepsy: Rationale and Clinical Perspectives

  2015cites this paper
• mTOR in Brain Physiology and Pathologies.

  2015influential citation
• Recessive nephrocerebellar syndrome on the Galloway-Mowat syndrome spectrum is caused by homozygous protein-truncating mutations of WDR73

  2015cites this paper
• LKB1 deletion causes early changes in atrial channel expression and electrophysiology prior to atrial fibrillation.

  2015cites this paper
• Epilepsy in Tuberous Sclerosis: Phenotypes, Mechanisms, and Treatments

  2015cites this paper
• A Genome-Wide siRNA Screen in Mammalian Cells for Regulators of S6 Phosphorylation

  2015cites this paper
• Pathogenesis and new candidate treatments for infantile spasms and early life epileptic encephalopathies: A view from preclinical studies.

  2015cites this paper
• mTOR signaling in epilepsy: insights from malformations of cortical development.

  2015cites this paper
• mTOR inhibition suppresses established epilepsy in a mouse model of cortical dysplasia

  2015cites this paper
• PI3K-AKT pathway polymerase chain reaction (PCR) array analysis of epilepsy induced by type II focal cortical dysplasia.

  2015cites this paper
• PI 3 K-AKT pathway polymerase chain reaction ( PCR ) array analysis of epilepsy induced by type II focal cortical dysplasia

  2015cites this paper
• Similar PDK1-AKT-mTOR pathway activation in balloon cells and dysmorphic neurons of type II focal cortical dysplasia with refractory epilepsy.

  2015influential citation
• Molecular Pathways Underlying Projection Neuron Production and Migration during Cerebral Cortical Development

  2015cites this paper
• Focal Cortical Dysplasia

  2014cites this paper
• Molecules and mechanisms that regulate multipolar migration in the intermediate zone

  2014cites this paper
• Hydrogen sulfide mitigates hyperglycemic remodeling via liver kinase B1-adenosine monophosphate-activated protein kinase signaling.

  2014cites this paper
• Day of the dead: pseudokinases and pseudophosphatases in physiology and disease.

  2014cites this paper
• mTOR

  2014cites this paper
• Phenotypes associated with inherited and developmental somatic mutations in genes encoding mTOR pathway components.

  2014cites this paper
• Rational Therapy from Bench to Bedside for a Rare Epilepsy

  2014influential citation
• Fetal brain mTOR signaling activation in tuberous sclerosis complex.

  2014cites this paper
• Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)

  2014cites this paper
• The relationship between genes affecting the development of epilepsy and approaches to epilepsy therapy

  2014cites this paper
• STRAD pseudokinases regulate axogenesis and LKB1 stability

  2014cites this paper
• Rheb Activation in Subventricular Zone Progenitors Leads to Heterotopia, Ectopic Neuronal Differentiation, and Rapamycin-Sensitive Olfactory Micronodules and Dendrite Hypertrophy of Newborn Neurons

  2013cites this paper
• Neurodevelopmental Disorder Rapamycin Prevents Seizures After Depletion of STRADA in a Rare

  2013cites this paper
• A critical review of mTOR inhibitors and epilepsy: from basic science to clinical trials

  2013cites this paper
• Rapamycin Prevents Seizures After Depletion of STRADA in a Rare Neurodevelopmental Disorder

  2013cites this paper
• mTOR inhibitors as a new therapeutic option for epilepsy

  2013cites this paper
• Role of the mTOR signaling pathway in epilepsy.

  2013cites this paper
• Mammalian Target of Rapamycin (mTOR) Pathways in Neurological Diseases

  2013cites this paper
• Dysregulation of mTOR activity through LKB1 inactivation

  2013cites this paper
• Mammalian target of rapamycin (mTOR) activation in focal cortical dysplasia and related focal cortical malformations.

  2013cites this paper
• Formation of chimeric genes by copy-number variation as a mutational mechanism in schizophrenia.

  2013cites this paper
• Focal malformations of cortical development: new vistas for molecular pathogenesis.

  2013cites this paper
• Acute inactivation of the serine-threonine kinase Stk25 disrupts neuronal migration

  2013influential citation
• Cancer Cell Metabolism

  2012cites this paper
• Amino acids and mTORC 1 : from lysosomes to disease

  2012cites this paper
• The role of TSC2 and deptor in fetal cortical development

  2012cites this paper
• Amino acids and mTORC1: from lysosomes to disease.

  2012cites this paper
• Multiple Roles for Mammalian Target of Rapamycin Signaling in Both Glutamatergic and GABAergic Synaptic Transmission

  2012cites this paper
• A homozygous missense mutation in HERC2 associated with global developmental delay and autism spectrum disorder

  2012cites this paper
• Mammalian Target of Rapamycin (mTOR) Inhibition: Potential for Antiseizure, Antiepileptogenic, and Epileptostatic Therapy

  2012cites this paper
• Finding a better drug for epilepsy: The mTOR pathway as an antiepileptogenic target

  2012cites this paper
• mTOR: A pathogenic signaling pathway in developmental brain malformations.

  2011cites this paper