Canavan disease and the role of N-acetylaspartate in myelin synthesis
Namboodiri AM, Peethambaran A, Mathew R, Sambhu PA, Hershfield J, Moffett JR, Madhavarao CN.
Mol Cell Endocrinol. 2006 Jun 27;252(1-2):216-23
In this article, the authors give further proof that N-acetylaspartate acts as an acetate carrier from mitochondria to the cytosol, and is thus important for lipogenesis and myelination in the central nervous system.
They recall that in the brain of Canavan disease mice (APSA -/-), there was nearly an 80% reduction in free acetate levels.
Supplementing these mice with glyceryl triacetate increases the brain acetate levels without affecting N-acetylaspartate levels and without toxicity.
For more information on Canavan disease, see chapter 229 of OMMBID.
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Philippe Campeau, MD
Resident in Medical Genetics at McGill University
OMMBID Blog Administrator