A deficit of brain dystrophin impairs specific amygdala GABAergic transmission and enhances defensive behaviour in mice.

No abstract is available for this paper.

• Publication year

  2009

• Venue

  Brain : a journal of neurology

• Publication date

  Unknown publication date

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1093/brain/awn253PMID 18927146
• 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.

• Central Nervous System

  2025cited by this paper
• Emotion circuits in the brain.

  2009cited by this paper
• GABAergic miniature spontaneous activity is increased in the CA1 hippocampal region of dystrophic mdx mice.

  2008cited by this paper
• Heightened Amygdala Long-Term Potentiation in Neurotensin Receptor Type-1 Knockout Mice

  2008influential reference
• Dystrophin levels as low as 30% are sufficient to avoid muscular dystrophy in the human.

  2007cited by this paper
• Evidence for the association of the S100β gene with low cognitive performance and dementia in the elderly

  2007cited by this paper
• Facilitation of Extinction Learning for Contextual Fear Memory by PEPA: A Potentiator of AMPA Receptors

  2007influential reference
• Impaired GABAergic transmission and altered hippocampal synaptic plasticity in collybistin‐deficient mice

  2007influential reference
• Postsynaptic targets of somatostatin‐containing interneurons in the rat basolateral amygdala

  2007cited by this paper
• Physiological and morphological characterization of parvalbumin‐containing interneurons of the rat basolateral amygdala

  2006influential reference
• Conditioned and unconditioned fear organized in the periaqueductal gray are differentially sensitive to injections of muscimol into amygdaloid nuclei.

  2006influential reference
• Composition and Assembly of Gabaergic Postsynaptic Specializations

  2006cited by this paper
• Effects of lesions to the dorsal and ventral hippocampus on defensive behaviors in rats

  2006influential reference
• Systemic delivery of morpholino oligonucleotide restores dystrophin expression bodywide and improves dystrophic pathology

  2006influential reference
• Molecular mechanisms of synaptogenesis

  2006cited by this paper
• Establishing an Agenda for Translational Research on PTSD

  2006cited by this paper
• Previous Stress Facilitates Fear Memory, Attenuates GABAergic Inhibition, and Increases Synaptic Plasticity in the Rat Basolateral Amygdala

  2005cited by this paper
• Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice.

  2004cited by this paper
• Impaired long-term spatial and recognition memory and enhanced CA1 hippocampal LTP in the dystrophin-deficient Dmd(mdx) mouse.

  2004cited by this paper
• Neurexins induce differentiation of GABA and glutamate postsynaptic specializations via neuroligins.

  2004cited by this paper
• Stress Impairs α1A Adrenoceptor-Mediated Noradrenergic Facilitation of GABAergic Transmission in the Basolateral Amygdala

  2004influential reference
• Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse

  2004cited by this paper
• Parvalbumin-containing interneurons in the basolateral amygdala express high levels of the alpha1 subunit of the GABAA receptor.

  2004cited by this paper
• Parvalbumin‐containing interneurons in the basolateral amygdala express high levels of the α1 subunit of the GABAA receptor

  2004cited by this paper
• Specific cognitive deficits are common in children with Duchenne muscular dystrophy.

  2004cited by this paper
• Amygdala stimulation modulates hippocampal synaptic plasticity.

  2004cited by this paper
• The amygdaloid complex: anatomy and physiology.

  2003influential reference
• Altered inhibitory input to Purkinje cells of dystrophin-deficient mice.

  2003cited by this paper
• Psychopathological assessment in children affected by Duchenne de Boulogne muscular dystrophy.

  2003cited by this paper
• The Mouse Defense Test Battery: pharmacological and behavioral assays for anxiety and panic.

  2003cited by this paper
• Role of bombesin (BN)-like peptides/receptors in emotional behavior by comparison of three strains of BN-like peptide receptor knockout mice

  2002influential reference
• GABAergic Terminals Are Required for Postsynaptic Clustering of Dystrophin But Not of GABAA Receptors and Gephyrin

  2002cited by this paper
• Dystroglycan Is Selectively Associated with Inhibitory GABAergic Synapses But Is Dispensable for Their Differentiation

  2002influential reference
• A stoichiometric complex of neurexins and dystroglycan in brain

  2001cited by this paper
• Collybistin, a newly identified brain-specific GEF, induces submembrane clustering of gephyrin

  2000cited by this paper
• Neurochemical mechanisms of the defensive behavior in the dorsal midbrain.

  1999cited by this paper
• Altered synaptic clustering of GABAA receptors in mice lacking dystrophin (mdx mice)

  1999influential reference
• Decreased GABAA-receptor clustering results in enhanced anxiety and a bias for threat cues

  1999cited by this paper
• Noradrenergic Excitation and Inhibition of GABAergic Cell Types in Rat Frontal Cortex

  1998influential reference
• Synapse‐specific localization of NMDA and GABAA receptor subunits revealed by antigen‐retrieval immunohistochemistry

  1998cited by this paper
• Dystrophin in the Nervous System

  1996influential reference
• Differential expression of dystrophin isoforms in strains of mdx mice with different mutations.

  1996cited by this paper
• Spatial learning and hippocampal long-term potentiation are not impaired in mdx mice.

  1996cited by this paper
• The mdx‐amplification‐resistant mutation system assay, a simple and rapid polymerase chain reaction‐based detection of the mdx allele

  1996cited by this paper
• Norepinephrine release in the amygdala in response to footshock stimulation.

  1996cited by this paper
• Duchenne muscular dystrophy

  1995cited by this paper
• Influence of dystrophin-gene mutation onmdx mouse behavior. I. Retention deficits at long delays in spontaneous alternation and bar-pressing tasks

  1995influential reference
• Synaptic plasticity in the basolateral amygdala induced by hippocampal formation stimulation in vivo

  1995influential reference
• mdx mice show progressive weakness and muscle deterioration with age.

  1995cited by this paper
• Cognitive impairment in Duchenne muscular dystrophy.

  1994cited by this paper
• The distribution of dystrophin in the murine central nervous system: an immunocytochemical study.

  1993cited by this paper
• Detection of dystrophin in the postsynaptic density of rat brain and deficiency in a mouse model of Duchenne muscular dystrophy.

  1992cited by this paper
• Glycoprotein‐binding site of dystrophin is confined to the cysteine‐rich domain and the first half of the carboxy‐terminal domain

  1992cited by this paper
• Cognitive functions in Duchenne muscular dystrophy: a reappraisal and comparison with spinal muscular atrophy.

  1992influential reference
• Comparative molecular neuroanatomy of cloned GABAA receptor subunits in the rat CNS

  1992cited by this paper
• Passive avoidance behaviour deficit in the mdx mouse.

  1991cited by this paper
• Membrane organization of the dystrophin-glycoprotein complex.

  1991cited by this paper
• Localization of dystrophin to postsynaptic regions of central nervous system cortical neurons

  1990influential reference
• The molecular basis of muscular dystrophy in the mdx mouse: a point mutation.

  1989cited by this paper
• Transcription of the dystrophin gene in human muscle and non-muscle tissues

  1988cited by this paper
• Different projections of the central amygdaloid nucleus mediate autonomic and behavioral correlates of conditioned fear

  1988cited by this paper
• Dystrophin: the protein product of the Duchenne muscular dystrophy locus.

  1987influential reference
• PSYCHIATRIC DISORDER AMONG BOYS WITH DUCHENNE MUSCULAR DYSTROPHY

  1986cited by this paper
• X chromosome-linked muscular dystrophy (mdx) in the mouse.

  1984influential reference
• Infantile autism and Duchenne muscular dystrophy

  1984cited by this paper
• Regional characteristics of stress-induced increases in brain noradrenaline release in rats.

  1983cited by this paper
• Innate and conditioned reactions to threat in rats with amygdaloid lesions.

  1972cited by this paper
• The central nervous system in Duchenne muscular dystrophy.

  1969cited by this paper
• The Central Nervous System

  1960cited by this paper

• Hyperactivity, compulsive-like behaviours, and impaired flexibility in mouse models of Duchenne muscular dystrophy.

  2026cites this paper
• Brain Matters in Duchenne Muscular Dystrophy: DMD Mutation Sites and Their Association with Neurological Comorbidities Through Isoform Impairment

  2025cites this paper
• Morpholino–RNA duplex exhibits robust, sustained, and safe steric-block antisense activity by intracerebroventricular and intrathecal injection

  2025cites this paper
• Brain dystrophinopathies and cognitive impairment: Pathogenesis, diagnosis, and therapies

  2025cites this paper
• Behavioral improvement in dystrophic mdx23 mouse following repeated antisense oligonucleotides injections

  2025cites this paper
• Considering the potential of ovarian hormones in rescuing neurological phenotypes in Duchenne muscular dystrophy – it’s time to think beyond the muscles

  2025cites this paper
• Regional Expression of Dystrophin Gene Transcripts and Proteins in the Mouse Brain

  2025cites this paper
• Understanding Duchenne muscular dystrophy-associated brain pathology

  2025cites this paper
• Beyond muscle: Delivering RNA therapeutics to the CNS in Duchenne muscular dystrophy

  2025cites this paper
• Electrophysiological Evaluation in mdx52 Mouse Brain After Antisense-Mediated Exon 51 or 53 Skipping.

  2025cites this paper
• Deficient Astrocyte Homeostatic Support Contributes to Brain Impairment in Duchenne Muscular Dystrophy

  2025cites this paper
• Obligatory and accessory respiratory muscle structure, function and control in early and advanced disease in the mdx mouse model of Duchenne muscular dystrophy

  2025cites this paper
• Neuronal hyperexcitability in dystrophin-deficient mdx hippocampal neurons: the importance of interleukin-6 and GABAergic regulation

  2025influential citation
• Stress-induced fetal programming contributes to the manifestation of Duchenne muscular dystrophy in mdx mice

  2025cites this paper
• Stress exposure in the mdx mouse model of Duchenne muscular dystrophy provokes a widespread metabolic response

  2025cites this paper
• Duchenne muscular dystrophy: recent insights in brain related comorbidities

  2025cites this paper
• Astrocyte proliferation in the hippocampal dentate gyrus is suppressed across the lifespan of dystrophin‐deficient mdx mice

  2025cites this paper
• The behavioural consequences of dystrophinopathy

  2025cites this paper
• The unconditioned fear response in vertebrates deficient in dystrophin.

  2024influential citation
• Cardio-metabolic and cytoskeletal proteomic signatures differentiate stress hypersensitivity in dystrophin-deficient mdx mice.

  2024cites this paper
• Inhibition of hippocampal interleukin-6 receptor-evoked signalling normalises long-term potentiation in dystrophin-deficient mdx mice

  2024cites this paper
• Impact of distinct dystrophin gene mutations on behavioral phenotypes of Duchenne muscular dystrophy

  2024cites this paper
• Brain glucose metabolism as a neuronal substrate of the abnormal behavioral response to stress in the mdx mouse, a model of Duchenne muscular dystrophy.

  2024influential citation
• The role of dystrophin isoforms and interactors in the brain

  2024influential citation
• Cerebellar dysfunction in the mdx mouse model of Duchenne muscular dystrophy: An electrophysiological and behavioural study

  2024cites this paper
• Heteroduplex oligonucleotide technology boosts oligonucleotide splice switching activity of morpholino oligomers in a Duchenne muscular dystrophy mouse model

  2024cites this paper
• Social and emotional alterations in mice lacking the short dystrophin-gene product, Dp71

  2024cites this paper
• Learning, memory and blood–brain barrier pathology in Duchenne muscular dystrophy mice lacking Dp427, or Dp427 and Dp140

  2024cites this paper
• AAV-Mediated Restoration of Dystrophin-Dp71 in the Brain of Dp71-Null Mice: Molecular, Cellular and Behavioral Outcomes

  2024cites this paper
• Retention of stress susceptibility in the mdx mouse model of Duchenne muscular dystrophy after PGC-1α overexpression or ablation of IDO1 or CD38.

  2024cites this paper
• Abnormality in GABAergic postsynaptic transmission associated with anxiety in Bronx waltzer mice with an Srrm4 mutation

  2023cites this paper
• Wybrane biopsychospołeczne aspekty funkcjonowania pacjentów z dystrofią mięśniową Duchenne’a i ich rodzin

  2023cites this paper
• Understanding anxiety experienced by young males with Duchenne muscular dystrophy: a qualitative focus group study.

  2023cites this paper
• The unconditioned fear response in dystrophin-deficient mice is associated with adrenal and vascular function

  2023cites this paper
• Partial restoration of brain dystrophin by tricyclo-DNA antisense oligonucleotides alleviates emotional deficits in mdx52 mice.

  2023cites this paper
• Brain magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy reveals regional reductions in cerebral gray matter

  2023cites this paper
• Dystrophinopathy and the brain: A parent project muscular dystrophy (PPMD) meeting report November 11-12, 2021, New York City, NY.

  2022cites this paper
• Brain Dp140 alters glutamatergic transmission and social behaviour in the mdx52 mouse model of Duchenne muscular dystrophy.

  2022influential citation
• Partial Restoration of Brain Dystrophin and Behavioral Deficits by Exon Skipping in the Muscular Dystrophy X‐Linked (mdx) Mouse

  2022influential citation
• Environmental, ecological and methodological factors of Tonic Immobility (TI) modulation.

  2022cites this paper
• Synaptic alterations as a neurodevelopmental trait of Duchenne muscular dystrophy.

  2022cites this paper
• Epileptic disorders in Becker and Duchenne muscular dystrophies: a systematic review and meta-analysis

  2022cites this paper
• Dystrophin Genotype and Risk of Neuropsychiatric Disorders in Dystrophinopathies: A Systematic Review and Meta-Analysis

  2022cites this paper
• Therapeutic Application of Extracellular Vesicles-Capsulated Adeno-Associated Virus Vector via nSMase2/Smpd3, Satellite, and Immune Cells in Duchenne Muscular Dystrophy

  2022cites this paper
• Retinal dystrophins and the retinopathy of Duchenne muscular dystrophy.

  2022cites this paper
• Validation of DE50-MD dogs as a model for the brain phenotype of Duchenne muscular dystrophy

  2022cites this paper
• Abnormal Expression of Synaptic and Extrasynaptic GABAA Receptor Subunits in the Dystrophin-Deficient mdx Mouse

  2022cites this paper
• Specific Dystrophins Selectively Associate with Inhibitory and Excitatory Synapses of the Mouse Cerebellum and their Loss Alters Expression of P2X7 Purinoceptors and Pro-Inflammatory Mediators

  2021cites this paper
• A new hypothesis of duchenne muscular dystrophy

  2021cites this paper
• Development and maintenance of vision's first synapse.

  2021cites this paper
• Novel Therapeutic Target of Duchenne Muscular Dystrophy: Circulating Exosomal microRNAs

  2021cites this paper
• Some dystrophy phenotypes of dystrophin‐deficient mdx mice are exacerbated by mild, repetitive daily stress

  2021cites this paper
• A New View on Duchenne Muscular Dystrophy

  2021cites this paper
• Palmitic acid conjugation enhances potency of tricyclo-DNA splice switching oligonucleotides

  2021cites this paper
• Sensitivity to behavioral stress impacts disease pathogenesis in dystrophin‐deficient mice

  2021cites this paper
• Dystrophin deficiency affects human astrocyte properties and response to damage

  2021cites this paper
• Deficiency of Glycosylated α-Dystroglycan in Ventral Hippocampus Bridges the Destabilization of Gamma-Aminobutyric Acid Type A Receptors With the Depressive-like Behaviors of Male Mice.

  2021cites this paper
• Disrupted Calcium Homeostasis in Duchenne Muscular Dystrophy: A Common Mechanism behind Diverse Consequences

  2021cites this paper
• Startle responses in Duchenne muscular dystrophy: a novel biomarker of brain dystrophin deficiency

  2021influential citation
• Emotional behavior and brain anatomy of the mdx52 mouse model of Duchenne muscular dystrophy

  2021influential citation
• Development of a novel startle response task in Duchenne muscular dystrophy

  2021cites this paper
• Assessment of Behavioral Characteristics With Procedures of Minimal Human Interference in the mdx Mouse Model for Duchenne Muscular Dystrophy

  2021influential citation
• Characterization of brain dystrophins absence and impact in dystrophin-deficient Dmdmdx rat model

  2020cites this paper
• Neurodevelopmental, behavioral, and emotional symptoms common in Duchenne muscular dystrophy

  2020cites this paper
• Early alterations of the behavioural structure of mice affected by Duchenne muscular dystrophy and tested in open-field.

  2020cites this paper
• Social stress is lethal in the mdx model of Duchenne muscular dystrophy

  2020cites this paper
• Long-Term Efficacy of AAV9-U7snRNA-Mediated Exon 51 Skipping in mdx52 Mice

  2020influential citation
• Interleukin-6: A neuro-active cytokine contributing to cognitive impairment in Duchenne muscular dystrophy?

  2020cites this paper
• 249th ENMC International Workshop: The role of brain dystrophin in muscular dystrophy: Implications for clinical care and translational research, Hoofddorp, The Netherlands, November 29th-December 1st 2019.

  2020influential citation
• Dystroglycan Mediates Clustering of Essential GABAergic Components in Cerebellar Purkinje Cells

  2020cites this paper
• Health-Related Quality of Life and Emotional Distress Among Mothers of Sons With Muscular Dystrophy as Compared to Sex- and Age Group–Matched Controls

  2020cites this paper
• The nSMase2/Smpd3 gene modulates the severity of muscular dystrophy and the emotional stress response in mdx mice

  2020cites this paper
• The role of brain dystrophin in muscular dystrophy: Implications for clinical care and translational research,

  2020cites this paper
• Inspiratory pressure‐generating capacity is preserved during ventilatory and non‐ventilatory behaviours in young dystrophic mdx mice despite profound diaphragm muscle weakness

  2019cites this paper
• Dystrophin Dp71 and the Neuropathophysiology of Duchenne Muscular Dystrophy

  2019cites this paper
• [The pathogenesis of Duchenne muscular dystrophy].

  2019cites this paper
• Gene Therapy for Central Nervous System in Duchenne Muscular Dystrophy

  2019cites this paper
• Inhibition in the amygdala anxiety circuitry

  2018cites this paper
• Influence of full-length dystrophin on brain volumes in mouse models of Duchenne muscular dystrophy

  2018cites this paper
• Prophylactic Bisphosphonate Therapy to Reduce Glucocorticoid-Induced Osteoporosis in a Mouse Model of Duchenne Muscular Dystrophy

  2018cites this paper
• Relationships linking emotional, motor, cognitive and GABAergic dysfunctions in dystrophin-deficient mdx mice

  2017cites this paper
• Decreased cerebral perfusion in Duchenne muscular dystrophy patients.

  2017cites this paper
• Investigating the brain in mouse models of Duchenne muscular dystrophy

  2017cites this paper
• Proteomic change by Korean Red Ginseng in the substantia nigra of a Parkinson's disease mouse model

  2017cites this paper
• Neurobiologie des troubles cognitifs des modèles murins de la myopathie de Duchenne

  2017cites this paper
• Common Hepatic Branch of Vagus Nerve-Dependent Expression of Immediate Early Genes in the Mouse Brain by Intraportal L-Arginine: Comparison with Cholecystokinin-8

  2017cites this paper
• Evaluation of the behavioral characteristics of the mdx mouse model of duchenne muscular dystrophy through operant conditioning procedures.

  2017cites this paper
• Efficacy and Safety Profile of Tricyclo-DNA Antisense Oligonucleotides in Duchenne Muscular Dystrophy Mouse Model

  2017cites this paper
• Localization and quantification of dystrophin in epileptic rats

  2016cites this paper
• Cognitive flexibility deficits in a mouse model for the absence of full‐length dystrophin

  2016cites this paper
• Reduced neuronal density in the CA1 anterodorsal hippocampus of the mdx mouse.

  2016cites this paper
• Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations

  2016cites this paper
• Cognitive dysfunction in Duchenne muscular dystrophy: a possible role for neuromodulatory immune molecules.

  2016cites this paper
• Genetic Determinism of Fearfulness, General Activity and Feeding Behavior in Chickens and Its Relationship with Digestive Efficiency

  2016cites this paper
• Translational Research on DMD in Japan

  2016cites this paper
• Evolving natural history in Duchenne muscular dystrophy : implications for standard of care and experimental therapies

  2016cites this paper
• Drug Discovery of Therapies for Duchenne Muscular Dystrophy

  2015cites this paper
• Acetylcholine, GABA and neuronal networks: a working hypothesis for compensations in the dystrophic brain.

  2015cites this paper
• Cognitive dysfunction in the dystrophin-deficient mouse model of Duchenne muscular dystrophy: A reappraisal from sensory to executive processes.

  2015cites this paper
• Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy

  2015cites this paper