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Article

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

The Journal of neuroscience : the official journal of the Society for Neuroscience - 28 Oct 2015

Maier Helena, Wang-Eckhardt Lihua, Hartmann Dieter, Gieselmann Volkmar, Eckhardt Matthias

Abstract excerpt

Canavan disease (CD) is a severe, lethal leukodystrophy caused by deficiency in aspartoacylase (ASPA), which hydrolyzes N-acetylaspartate (NAA). In the brains of CD patients, NAA accumulates to high millimolar concentrations. The pathology of the disease is characterized by loss of oligodendrocytes and spongy myelin degeneration in the CNS. Whether accumulating NAA, absence of NAA-derived acetate, or absence of...

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Source

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Identifiers

PMID: 26511242
DOI: 10.1523/JNEUROSCI.1056-15.2015
PMCID: PMC6605458

Genes

ASPA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time.PMID 26511242Read ↗

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