N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time. (Q40382537)

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28 February 2020

title

N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26511242%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

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Helena Maier

1

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28 February 2020

Lihua Wang-Eckhardt

2

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28 February 2020

Dieter Hartmann

3

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28 February 2020

Volkmar Gieselmann

4

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28 February 2020

Matthias Eckhardt

5

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28 February 2020

language of work or name

English

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publication date

1 October 2015

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28 February 2020

published in

Journal of Neuroscience

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28 February 2020

volume

35

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28 February 2020

page(s)

14501-14516

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28 February 2020

issue

43

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

28 February 2020

cites work

1 reference

12 December 2020

Absence of N-acetylaspartate in the human brain: impact on neurospectroscopy?

1 reference

12 December 2020

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

1 reference

12 December 2020

N-acetylaspartate is an axon-specific marker of mature white matter in vivo: a biochemical and immunohistochemical study on the rat optic nerve

1 reference

12 December 2020

N-acetylaspartate in the vertebrate brain: metabolism and function

1 reference

12 December 2020

Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain

1 reference

12 December 2020

Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse.

1 reference

12 December 2020

Delay of myelin formation in arylsulphatase A-deficient mice

1 reference

12 December 2020

Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease.

1 reference

12 December 2020

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

1 reference

12 December 2020

A new mouse embryonic stem cell line with good germ line contribution and gene targeting frequency

1 reference

12 December 2020

Canavan disease: studies on the knockout mouse

1 reference

12 December 2020

Physiological role of N-acetylaspartate: contribution to myelinogenesis

1 reference

12 December 2020

Defective myelin lipid synthesis as a pathogenic mechanism of Canavan disease

1 reference

12 December 2020

N-Acetylaspartate in the CNS: from neurodiagnostics to neurobiology

1 reference

12 December 2020

Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2.

1 reference

12 December 2020

Myelin lipid abnormalities in the aspartoacylase-deficient tremor rat.

1 reference

12 December 2020

Can the choice of intermediate solvent or resin affect glomerular basement membrane thickness?

1 reference

12 December 2020

Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration

1 reference

12 December 2020

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

1 reference

12 December 2020

Molecular identification of aspartate N-acetyltransferase and its mutation in hypoacetylaspartia

1 reference

12 December 2020

Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease

1 reference

12 December 2020

Molecular characterization of N-acetylaspartylglutamate synthetase

1 reference

12 December 2020

Molecular identification of N-acetylaspartylglutamate synthase and beta-citrylglutamate synthase

1 reference

12 December 2020

N-acetylaspartylglutamate synthetase II synthesizes N-acetylaspartylglutamylglutamate

1 reference

12 December 2020

Extensive aspartoacylase expression in the rat central nervous system

1 reference

12 December 2020

Aspartoacylase-lacZ knockin mice: an engineered model of Canavan disease

1 reference

12 December 2020

Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions

1 reference

12 December 2020

A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice.

1 reference

12 December 2020

NAT8L (N-acetyltransferase 8-like) accelerates lipid turnover and increases energy expenditure in brown adipocytes

1 reference

12 December 2020

Canavan disease: clinical features and recent advances in research.

1 reference

12 December 2020

Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.

1 reference

12 December 2020

Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with canavan disease

1 reference

12 December 2020

N-Acetylation of L-aspartate in the nervous system: differential distribution of a specific enzyme.

1 reference

12 December 2020

Acetate metabolism in the nervous system. N-acetyl-L-aspartic acid and the biosynthesis of brain lipids.

1 reference

12 December 2020

Detection of increased urinary N-acetylaspartylglutamate in Canavan disease

1 reference

12 December 2020

10.1523/JNEUROSCI.1056-15.2015

Identifiers

DOI

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28 February 2020

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