Non-genetic therapeutic approaches to Canavan disease.

No abstract is available for this paper.

• Publication year

  2016

• Venue

  Journal of Neurological Sciences

• Publication date

  2016-07-15

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/j.jns.2016.05.012PMID 27288788
• 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.

• Canavan disease: an Arab scenario.

  2015cited by this paper
• Ablating N‐acetylaspartate prevents leukodystrophy in a Canavan disease model

  2015cited by this paper
• Canavan disease: Clinical features and recent advances in research

  2014cited by this paper
• Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease

  2014influential reference
• Lithium and GSK3-β Promoter Gene Variants Influence White Matter Microstructure in Bipolar Disorder

  2013cited by this paper
• Advances and Pitfalls of Cell Therapy in Metabolic Leukodystrophies

  2013cited by this paper
• Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions.

  2013influential reference
• Potential Mechanisms of Action of Lithium in Bipolar Disorder

  2013cited by this paper
• A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice.

  2013influential reference
• Aspartoacylase Supports Oxidative Energy Metabolism during Myelination

  2012cited by this paper
• Long-Term Follow-Up After Gene Therapy for Canavan Disease

  2012cited by this paper
• Lithium citrate as treatment of Canavan disease.

  2012cited by this paper
• Lithium enhances remyelination of peripheral nerves

  2012cited by this paper
• Upregulation of N-acetylaspartic Acid Induces Oxidative Stress to Contribute in Disease Pathophysiology

  2011cited by this paper
• A safety trial of high dose glyceryl triacetate for Canavan disease.

  2011influential reference
• Neuroprotective role of lipoic acid against acute toxicity of N-acetylaspartic acid

  2010cited by this paper
• Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease

  2010influential reference
• Lithium citrate reduces excessive intra-cerebral N-acetyl aspartate in Canavan disease.

  2010cited by this paper
• Donor-Derived Brain Tumor Following Neural Stem Cell Transplantation in an Ataxia Telangiectasia Patient

  2009cited by this paper
• Are Astrocytes the Missing Link Between Lack of Brain Aspartoacylase Activity and the Spongiform Leukodystrophy in Canavan Disease?

  2009cited by this paper
• Glyceryl triacetate for Canavan disease: A low-dose trial in infants and evaluation of a higher dose for toxicity in the tremor rat model

  2009influential reference
• Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats

  2009cited by this paper
• Canavan disease

  2008cited by this paper
• The astrocyte surface NAAG receptor and NAAG peptidase signaling complex as a therapeutic target.

  2008cited by this paper
• Myelin Lipid Abnormalities in the Aspartoacylase-Deficient Tremor Rat

  2008cited by this paper
• N-Acetylaspartate and N-acetylaspartylglutamate

  2008cited by this paper
• N‐Acetylaspartic acid promotes oxidative stress in cerebral cortex of rats

  2007cited by this paper
• N-Acetylaspartate in the CNS: from neurodiagnostics to neurobiology.

  2007cited by this paper
• Homozygosity for mutation G212A of the gene for aspartoacylase is associated with atypical form of Canavan’s disease

  2007cited by this paper
• Brain N-acetylaspartate as a molecular water pump and its role in the etiology of canavan disease

  2007cited by this paper
• Bimodal occurrence of aspartoacylase in myelin and cytosol of brain

  2007cited by this paper
• Mild‐onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase gene

  2006cited by this paper
• Canavan disease and the role of N-acetylaspartate in myelin synthesis.

  2006influential reference
• Natural History of Canavan Disease Revealed by Proton Magnetic Resonance Spectroscopy (1H‐MRS) and Diffusion-weighted MRI

  2006cited by this paper
• Canavan disease: a white matter disorder.

  2006cited by this paper
• Lithium citrate for Canavan disease.

  2005influential reference
• Effects of AAV-2-mediated aspartoacylase gene transfer in the tremor rat model of Canavan disease.

  2005cited by this paper
• Progress toward Acetate Supplementation Therapy for Canavan Disease: Glyceryl Triacetate Administration Increases Acetate, but Not N-Acetylaspartate, Levels in Brain

  2005influential reference
• Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease.

  2005cited by this paper
• Canavan disease: Carrier‐frequency determination in the Ashkenazi Jewish population and development of a novel molecular diagnostic assay

  2004cited by this paper
• Effect of topiramate on enlargement of head in Canavan disease: a new option for treatment of megalencephaly.

  2004cited by this paper
• Immunohistochemical localization of aspartoacylase in the rat central nervous system

  2004cited by this paper
• Mouse neural progenitor cells differentiate into oligodendrocytes in the brain of a knockout mouse model of Canavan disease.

  2004influential reference
• Human Gene Mutation Database (HGMD®): 2003 update

  2003cited by this paper
• Identification and distribution of aspartoacylase in the postnatal rat brain

  2003cited by this paper
• Developmental increase of aspartoacylase in oligodendrocytes parallels CNS myelination.

  2003cited by this paper
• Chronic lithium and sodium valproate both decrease the concentration of myoinositol and increase the concentration of inositol monophosphates in rat brain.

  2003cited by this paper
• Molecular Basis of Canavan's Disease

  2003cited by this paper
• Aspartoacylase is restricted primarily to myelin synthesizing cells in the CNS: therapeutic implications for Canavan disease.

  2002cited by this paper
• The Effects of Lithium Chloride and Other Substances on Levels of Brain N-Acetyl-L-Aspartic Acid in Canavan Disease-Like Rats

  2002cited by this paper
• A radiometric assay for aspartoacylase activity in cultured oligodendrocytes.

  2002cited by this paper
• Developmental and regional distribution of aspartoacylase in rat brain tissue

  2001cited by this paper
• Aspartoacylase gene transfer to the mammalian central nervous system with therapeutic implications for Canavan disease

  2000cited by this paper
• Accumulation of N‐Acetyl‐L‐Aspartate in the Brain of the Tremor Rat, a Mutant Exhibiting Absence‐Like Seizure and Spongiform Degeneration in the Central Nervous System

  2000cited by this paper
• Mutation detection in the aspartoacylase gene in 17 patients with Canavan disease: four new mutations in the non-Jewish population

  2000cited by this paper
• Chronic lithium and sodium valproate both decrease the concentration of myo-inositol and increase the concentration of inositol monophosphates in rat brain.

  2000cited by this paper
• Canavan’s spongiform leukodystrophy

  2000cited by this paper
• Effects of ethanol and of alcohol dehydrogenase inhibitors on the reduction of N-acetylaspartate levels of brain in mice in vivo: a search for substances that may have therapeutic value in the treatment of Canavan disease

  2000cited by this paper
• Expression of aspartoacylase activity in cultured rat macroglial cells is limited to oligodendrocytes

  1999cited by this paper
• Global CNS gene transfer for a childhood neurogenetic enzyme deficiency: Canavan disease.

  1999cited by this paper
• Biochemistry and Molecular Biology of Canavan Disease

  1999cited by this paper
• Recent advances in Canavan disease.

  1999cited by this paper
• Brain Adaptation to Water Loading in Rabbits as Assessed by NMR Relaxometry

  1999cited by this paper
• Spongy degeneration of the brain, Canavan disease: biochemical and molecular findings.

  1998cited by this paper
• The clinical course of Canavan disease.

  1998cited by this paper
• Transient Elevation of Interstitial N‐Acetylaspartate in Reversible Global Brain Ischemia

  1997cited by this paper
• Canavan disease

  1997cited by this paper
• Identification and expression of eight novel mutations among non-Jewish patients with Canavan disease.

  1996cited by this paper
• Canavan disease: from spongy degeneration to molecular analysis.

  1995cited by this paper
• Differential expression of metabotropic glutamate receptors in the hippocampus and entorhinal cortex of the rat.

  1994cited by this paper
• MRI and MRS studies on acute effects of ethanol in the rat brain.

  1994cited by this paper
• Canavan disease: mutations among Jewish and non-Jewish patients.

  1994cited by this paper
• Cloning of the human aspartoacylase cDNA and a common missense mutation in Canavan disease

  1993cited by this paper
• Purification, Characterization, and Localization of Aspartoacylase from Bovine Brain

  1991cited by this paper
• Infantile CNS spongy degeneration‐14 cases

  1990cited by this paper
• Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan disease.

  1988cited by this paper
• N-acetylaspartic aciduria due to aspartoacylase deficiency — a new aetiology of childhood leukodystrophy

  1987cited by this paper
• Spongy degeneration of the central nervous system (Van Bogaert-Bertrand type?) in a newborn infant

  1977cited by this paper
• Spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan's disease). A review.

  1973cited by this paper
• Cerebral spongy degeneration of infancy

  1972cited by this paper
• Protracted form of spongy degeneration of the central nervous system: (van Bogaert and Bertrand type)

  1968cited by this paper
• ACETATE METABOLISM IN THE NERVOUS SYSTEM. N‐ACETYL‐l‐ASPARTIC ACID AND THE BIOSYNTHESIS OF BRAIN LIPIDS *

  1966cited by this paper
• [Schilder's encephalitis periaxialis diffusa].

  1950cited by this paper
• PROGRESSIVE DEGENERATIVE SUBCORTICAL ENCEPHALOPATHY: (SCHILDER'S DISEASE)

  1928cited by this paper

• An Overview of Oligodendrocyte Metabolism.

  2025cites this paper
• Deep mutational scanning reveals a correlation between degradation and toxicity of thousands of aspartoacylase variants

  2024cites this paper
• Cellular and molecular mechanisms of aspartoacylase and its role in Canavan disease

  2024influential citation
• Deep mutational scanning reveals a tight correlation between protein degradation and toxicity of thousands of non-native aspartoacylase protein variants

  2023cites this paper
• The pathogenesis of, and pharmacological treatment for, Canavan disease.

  2022cites this paper
• mTOR-Rictor-EGFR axis in oncogenesis and diagnosis of glioblastoma multiforme

  2021cites this paper
• Embryology, Malformations, and Rare Diseases of the Cochlea

  2021cites this paper
• Disorders of myelin

  2020cites this paper
• Antisense Oligonucleotide Reverses Leukodystrophy in Canavan Disease Mice

  2020influential citation
• Canavan Disease: Clinical and Laboratory Profile from Southern Part of India

  2020cites this paper
• A case of juvenile Canavan disease with distinct pons involvement.

  2019cites this paper
• Opening New Horizons in the Treatment of Childhood Onset Leukodystrophies

  2019cites this paper
• Brain N-acetylaspartate accumulation in Canavan disease is not neurotoxic per se: the implications of the first gene replacement therapy study to demonstrate successful post-symptomatic treatment in mice

  2018cites this paper
• Viral Vector Gene Delivery to the Brain for Treating Neurogenetic Diseases

  2017cites this paper
• Microbial Neuraminidase Induces a Moderate and Transient Myelin Vacuolation Independent of Complement System Activation

  2017cites this paper
• Drug and Gene Delivery to the Central Nervous System for Neuroprotection

  2017cites this paper
• Cytotoxic edema and diffusion restriction as an early pathoradiologic marker in canavan disease: case report and review of the literature

  2016cites this paper