Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease.

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Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease. is …
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scholarly articleQ13442814

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P356DOI10.1172/JCI.INSIGHT.90807
P932PMC publication ID5291725
P698PubMed publication ID28194442

P50authorDanning LiQ89267923
P2093author name stringQin Su
Guangping Gao
Jean King
Julio Sanmiguel
Reuben Matalon
Hongxia Xu
Dominic J Gessler
Constance Moore
Serafettin Tuncer
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A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice.Q30559496
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Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease.Q33933163
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rAAV Gene Therapy in a Canavan's Disease Mouse Model Reveals Immune Impairments and an Extended Pathology Beyond the Central Nervous System.Q37043518
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Are astrocytes the missing link between lack of brain aspartoacylase activity and the spongiform leukodystrophy in Canavan disease?Q37423187
N-acetylaspartate (NAA) induces neuronal differentiation of SH-SY5Y neuroblastoma cell line and sensitizes it to chemotherapeutic agentsQ38781562
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Absence of SHATI/Nat8l reduces social interaction in mice.Q39287252
Spongy degeneration of the central nervous system (Van Bogaert and Bertrand type; Canavan's disease)A reviewQ39938567
Characterization of astrocyte-specific conditional knockoutsQ40141365
N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time.Q40382537
CT and MR imaging of Canavan diseaseQ42052033
Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytesQ42739394
Glyceryl triacetate for Canavan disease: a low-dose trial in infants and evaluation of a higher dose for toxicity in the tremor rat modelQ43291024
Resting state fMRI reveals increased subthalamic nucleus-motor cortex connectivity in Parkinson's disease.Q43912490
Effects of AAV-2-mediated aspartoacylase gene transfer in the tremor rat model of Canavan diseaseQ44054737
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Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease.Q44719583
Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan diseaseQ45090529
In vivo quantitation of cerebral metabolite concentrations using natural abundance 13C MRS at 1.5 T.Q45297254
Variable course of Canavan disease in two boys with early infantile aspartoacylase deficiencyQ45749661
Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.Q45934574
Progress toward acetate supplementation therapy for Canavan disease: glyceryl triacetate administration increases acetate, but not N-acetylaspartate, levels in brainQ46588627
Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with canavan diseaseQ48115232
Reduced hippocampal N-acetylaspartate without volume loss in schizophreniaQ48165321
Automatic quantitation of localized in vivo 1H spectra with LCModelQ48842365
N-Acetyl-L-aspartic acid in brain.Q51332544
The clinical course of Canavan disease.Q51994919
Glial Glycolysis Is Essential for Neuronal Survival in Drosophila.Q52812695
Magnetic resonance imaging in juvenile Canavan diseaseQ61735501
Expression of aspartoacylase activity in cultured rat macroglial cells is limited to oligodendrocytesQ73484896
P4510describes a project that usesImageJQ1659584
P433issue3
P921main subjectmyelinationQ14876135
P304page(s)e90807
P577publication date2017-02-09
P1433published inJCI insightQ27727187
P1476titleRedirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease
P478volume2

Reverse relations

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Q33753200Increasing N-acetylaspartate in the Brain during Postnatal Myelination Does Not Cause the CNS Pathologies of Canavan Disease.
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