Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS - Wikidata - wikimedia - TriplyDB

Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS

Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS

http://www.wikidata.org/entity/Q30485283

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Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease Aspartoacylase-lacZ knockin mice: an engineered model of Canavan disease Involvement of aspartoacylase in tremor expression in rats.Localisation of N-acetylaspartate in oligodendrocytes/myelin ZFP191 is required by oligodendrocytes for CNS myelination A new mouse model of Canavan leukodystrophy displays hearing impairment due to central nervous system dysmyelination Concurrent Lpin1 and Nrcam mouse mutations result in severe peripheral neuropathy with transitory hindlimb paralysis.A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice.A genetic mouse model of adult-onset, pervasive central nervous system demyelination with robust remyelination.Discovery of candidate disease genes in ENU-induced mouse mutants by large-scale sequencing, including a splice-site mutation in nucleoredoxin.Increasing N-acetylaspartate in the Brain during Postnatal Myelination Does Not Cause the CNS Pathologies of Canavan Disease.Pathologic and phenotypic alterations in a mouse expressing a connexin47 missense mutation that causes Pelizaeus-Merzbacher-like disease in humans.Gata6 regulates aspartoacylase expression in resident peritoneal macrophages and controls their survival.The energetics of CNS white matter.Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.Extensive aspartoacylase expression in the rat central nervous system.Loss of central auditory processing in a mouse model of Canavan disease.A mouse model of L-2-hydroxyglutaric aciduria, a disorder of metabolite repair.Transcriptional regulation of N-acetylaspartate metabolism in the 5xFAD model of Alzheimer's disease: evidence for neuron-glia communication during energetic crisis.Making the White Matter Matters: Progress in Understanding Canavan's Disease and Therapeutic Interventions Through Eight Decades.Clinical applications involving CNS gene transfer.Oligodendrocyte death results in immune-mediated CNS demyelination.Adenomatous polyposis coli regulates oligodendroglial development.Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders.Suppressing N-Acetyl-l-Aspartate Synthesis Prevents Loss of Neurons in a Murine Model of Canavan Leukodystrophy A thematic review of scientific and family interests in Canavan Disease: where are the developmentalists?Endogenous aspartoacylase expression is responsive to glutamatergic activity in vitro and in vivo.Inherited and acquired disorders of myelin: The underlying myelin pathology.Short-term cuprizone feeding verifies N-acetylaspartate quantification as a marker of neurodegeneration.N-acetylaspartate supports the energetic demands of developmental myelination via oligodendroglial aspartoacylase.The neuronal metabolite NAA regulates histone H3 methylation in oligodendrocytes and myelin lipid composition.Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.Aspartoacylase supports oxidative energy metabolism during myelination Astroglial redistribution of aquaporin 4 during spongy degeneration in a Canavan disease mouse model.Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease.Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.Conditional knockout of TOG results in CNS hypomyelination.Brain Nat8l Knockdown Suppresses Spongiform Leukodystrophy in an Aspartoacylase-Deficient Canavan Disease Mouse Model.

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P2860

Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS

http://www.wikidata.org/entity/Q30485283

description

2008 nî lūn-bûn

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2008 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած

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2008 թվականի նոյեմբերին հրատարակված գիտական հոդված

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2008年の論文

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Nur7 is a nonsense mutation in ...... spongy degeneration of the CNS

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P2093

Ben A Barres

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Jason Dugas

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Maria Traka

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Robert L Wollmann

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Sonia R Cerda

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P2860

Cloning of the human aspartoacylase cDNA and a common missense mutation in Canavan disease

A simple method for the isolation and purification of total lipides from animal tissues

N-Acetylaspartylglutamate: the most abundant peptide neurotransmitter in the mammalian central nervous system

Functional genetic analysis of mouse chromosome 11

Intraneuronal N-acetylaspartate supplies acetyl groups for myelin lipid synthesis: evidence for myelin-associated aspartoacylase

Aspartoacylase is a regulated nuclear-cytoplasmic enzyme

Developmental increase of aspartoacylase in oligodendrocytes parallels CNS myelination

The cytoplasmic domain of the large myelin-associated glycoprotein isoform is needed for proper CNS but not peripheral nervous system myelination

A comparison of cell and tissue extraction techniques using high-resolution 1H-NMR spectroscopy.

Knock-out mouse for Canavan disease: a model for gene transfer to the central nervous system.

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