Mutation of ataxia–telangiectasia mutated is associated with dysfunctional glutathione homeostasis in cerebellar astroglia

No abstract is available for this paper.

• Publication year

  2016

• Venue

  Glia

• Publication date

  2016-02-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1002/glia.22925PMID 26469940PMCID PMC5580048
• 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.

• Ataxia-Telangiectasia

  2020cited by this paper
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• Chromosome Instability and Oxidative Stress Markers in Patients with Ataxia Telangiectasia and Their Parents

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• Astrogliopathy and oligodendrogliopathy are early events in CNS demyelination

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• A randomized double blind placebo controlled clinical trial of N-Acetylcysteine added to risperidone for treating autistic disorders

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• Aβ induces astrocytic glutamate release, extrasynaptic NMDA receptor activation, and synaptic loss

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• Microdialysis Study of Cefotaxime Cerebral Distribution in Patients with Acute Brain Injury

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• Pathogenesis of ataxia-telangiectasia: the next generation of ATM functions.

  2013cited by this paper
• The Cystine/Glutamate Antiporter System xc − in Health and Disease: From Molecular Mechanisms to Novel Therapeutic Opportunities

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• How many neurons do you have? Some dogmas of quantitative neuroscience under revision

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• Nuclear accumulation of HDAC4 in ATM deficiency promotes neurodegeneration in ataxia-telangiectasia

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• Role of Cell Cycle Re-Entry in Neurons: A Common Apoptotic Mechanism of Neuronal Cell Death

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  2012cited by this paper
• Activation of AMP-activated protein kinase in cerebella of Atm-/- mice is attributable to accumulation of reactive oxygen species.

  2012cited by this paper
• Transplantation of Specific Human Astrocytes Promotes Functional Recovery after Spinal Cord Injury

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• Ataxia telangiectasia: A report of two cousins and review of literature

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• Lower whole blood glutathione peroxidase (GPX) activity in depression, but not in myalgic encephalomyelitis / chronic fatigue syndrome: another pathway that may be associated with coronary artery disease and neuroprogression in depression.

  2011cited by this paper
• Loss of System xc− Does Not Induce Oxidative Stress But Decreases Extracellular Glutamate in Hippocampus and Influences Spatial Working Memory and Limbic Seizure Susceptibility

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• Wnt/β-catenin Signalling Is Active in a Highly Dynamic Pattern during Development of the Mouse Cerebellum

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  2011cited by this paper
• Regulation of the System x−C cystine/glutamate exchanger by intracellular glutathione levels in rat astrocyte primary cultures

  2011influential reference
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• ATM Activation by Oxidative Stress

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  2010cited by this paper
• Controlled enzymatic production of astrocytic hydrogen peroxide protects neurons from oxidative stress via an Nrf2-independent pathway

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• Autopsy study of cerebellar degeneration in siblings with ataxia-telangiectasia-like disorder

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• Induction of Nrf2 and xCT are involved in the action of the neuroprotective antibiotic ceftriaxone in vitro

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• Astrocytic Dysfunction in Epileptogenesis: Consequence of Altered Potassium and Glutamate Homeostasis?

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• Nrf2-mediated neuroprotection in the MPTP mouse model of Parkinson's disease: Critical role for the astrocyte

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• Oxidative Stress Is Linked to ERK1/2-p16 Signaling-mediated Growth Defect in ATM-deficient Astrocytes*

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• Equal numbers of neuronal and nonneuronal cells make the human brain an isometrically scaled‐up primate brain

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• Brain MRI Abnormalities in Ataxia-Telangiectasia

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• Experimental Antioxidant Therapy in Ataxia Telangiectasia

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• Transplanted astrocytes derived from BMP- or CNTF-treated glial-restricted precursors have opposite effects on recovery and allodynia after spinal cord injury

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• Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

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• Identification of new kinase clusters required for neurite outgrowth and retraction by a loss-of-function RNA interference screen

  2008cited by this paper
• Regulation of Reactive Oxygen Species by Atm Is Essential for Proper Response to DNA Double-Strand Breaks in Lymphocytes1

  2007cited by this paper
• ATM and ATR Substrate Analysis Reveals Extensive Protein Networks Responsive to DNA Damage

  2007cited by this paper
• Intrinsic mitochondrial dysfunction in ATM-deficient lymphoblastoid cells.

  2007cited by this paper
• Inhibition of ataxia telangiectasia-p53-E2F-1 pathway in neurons as a target for the prevention of neuronal apoptosis.

  2007cited by this paper
• Cystine/Glutamate Exchange Modulates Glutathione Supply for Neuroprotection from Oxidative Stress and Cell Proliferation

  2006influential reference
• Alexander Disease-Associated Glial Fibrillary Acidic Protein Mutations in Mice Induce Rosenthal Fiber Formation and a White Matter Stress Response

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• Separation and quantitation of water soluble cellular metabolites by hydrophilic interaction chromatography-tandem mass spectrometry.

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• Essential protective roles of reactive astrocytes in traumatic brain injury.

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• Reactive Astrocytes in Neural Repair and Protection

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• ATM deficiency induces oxidative stress and endoplasmic reticulum stress in astrocytes

  2005influential reference
• Redox Imbalance in Cystine/Glutamate Transporter-deficient Mice*

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• Impaired genomic stability and increased oxidative stress exacerbate different features of Ataxia-telangiectasia.

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• Co-culture of neurones with glutathione deficient astrocytes leads to increased neuronal susceptibility to nitric oxide and increased glutamate-cysteine ligase activity.

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• Ataxia-telangiectasia: the pattern of cerebellar atrophy on MRI

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• Role of oxidative stress in telomere shortening in cultured fibroblasts from normal individuals and patients with ataxia-telangiectasia.

  2003cited by this paper
• Coordinate Regulation of Glutathione Biosynthesis and Release by Nrf2-Expressing Glia Potently Protects Neurons from Oxidative Stress

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• Oxidative Stress Is Responsible for Deficient Survival and Dendritogenesis in Purkinje Neurons from Ataxia-Telangiectasia Mutated Mutant Mice

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• Elevated Oxidative Stress in Patients with Ataxia Telangiectasia

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• Increased oxidative stress in ataxia telangiectasia evidenced by alterations in redox state of brains from Atm-deficient mice.

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• Superoxide stress identifies neurons at risk in a model of ataxia‐telangiectasia

  2001cited by this paper
• Astrocytes Provide Cysteine to Neurons by Releasing Glutathione

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• Glutathione metabolism in brain

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• Metabolism and functions of glutathione in brain.

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• Ataxia telangiectasia mutated deficiency affects astrocyte growth but not radiosensitivity.

  1999cited by this paper
• N-acetylcysteine, but not methionine or 2-oxothiazolidine-4-carboxylate, serves as cysteine donor for the synthesis of glutathione in cultured neurons derived from embryonal rat brain.

  1999cited by this paper
• Astrocytes prevent neuronal death induced by reactive oxygen and nitrogen species

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• Loss of the ataxia-telangiectasia gene product causes oxidative damage in target organs.

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• Astrocytes: Glutamate producers for neurons

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• Utilization of cysteine and cysteine precursors for the synthesis of glutathione in astroglial cultures: Preference for cystine

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• Ataxia-telangiectasia: is ATM a sensor of oxidative damage and stress?

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• The γ-Glutamyl Transpeptidase Inhibitor Acivicin Preserves Glutathione Released by Astroglial Cells in Culture

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• Atm-deficient mice: a paradigm of ataxia telangiectasia.

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• Loss of CD98HC phosphorylation by ATM impairs antiporter trafficking and drives glutamate toxicity in Ataxia telangiectasia

  2025cites this paper
• Hydrogen Sulfide Signaling in Neurodegenerative Movement Disorders.

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• Computational modeling of ATM signaling: a predictive framework for drug repurposing in ataxia-telangiectasia

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• Pioglitazone as a Possible Treatment for Ataxia-Telangiectasia

  2024influential citation
• ATM phosphorylation of CD98HC increases antiporter membrane localization and prevents chronic toxic glutamate accumulation in Ataxia telangiectasia

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• Pioglitazone treatment increases the cellular acid-labile and protein-bound sulfane sulfur fractions.

  2023cites this paper
• New Views of the DNA Repair Protein Ataxia–Telangiectasia Mutated in Central Neurons: Contribution in Synaptic Dysfunctions of Neurodevelopmental and Neurodegenerative Diseases

  2023cites this paper
• The ataxia-telangiectasia mutated gene product regulates the cellular acid-labile sulfide fraction.

  2022cites this paper
• A novel, ataxic mouse model of ataxia telangiectasia caused by a clinically relevant nonsense mutation

  2020cites this paper
• Deletion of P2X7 Receptor Decreases Basal Glutathione Level by Changing Glutamate-Glutamine Cycle and Neutral Amino Acid Transporters

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• Cerebellar Astrocytes: Much More Than Passive Bystanders In Ataxia Pathophysiology

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• The Role of Astrocytes in the Development of the Cerebellum

  2019cites this paper
• Inactive Atm abrogates DSB repair in mouse cerebellum more than does Atm loss, without causing a neurological phenotype.

  2018cites this paper
• Neurodegeneration in ataxia‐telangiectasia: Multiple roles of ATM kinase in cellular homeostasis

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• ATM-dependent pathways of chromatin remodelling and oxidative DNA damage responses

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• Dexamethasone improves redox state in ataxia telangiectasia cells by promoting an NRF2‐mediated antioxidant response

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