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Article

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

Journal of inherited metabolic disease - 1 Jun 2010

Arun Peethambaran, Madhavarao Chikkathur N, Moffett John R, Hamilton Kristen, Grunberg Neil E, Ariyannur Prasanth S, Gahl William A, Anikster Yair, Mog Steven, Hallows William C, Denu John M, Namboodiri Aryan M A

Abstract excerpt

Genetic mutations that severely diminish the activity of aspartoacylase (ASPA) result in the fatal brain dysmyelinating disorder, Canavan disease. There is no effective treatment. ASPA produces free acetate from the concentrated brain metabolite, N-acetylaspartate (NAA). Because acetyl coenzyme A is a key building block for lipid synthesis, we postulated that the inability to catabolize NAA leads to a brain...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 20464498
DOI: 10.1007/s10545-010-9100-z
PMCID: PMC2877317

Genes

ASPA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

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Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease.PMID 20464498Read ↗

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