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DOI: 10.1055/s-0031-1277176
© Georg Thieme Verlag KG Stuttgart · New York
N-Acetylaspartylglutamate in CNS Hypomyelination
Publication History
received 10.03.2011
accepted 12.04.2011
Publication Date:
03 May 2011 (online)

Abstract
CSF N-acetylaspartylglutamate (NAAG) has been found to be elevated in some hypomyelinating disorders. This study addressed the question whether it could be used as a marker for hypomyelination and as a means to distinguish between hypomyelinating disorders biochemically. We have measured CSF NAAG in a cohort of 28 patients with hypomyelination with known and unknown aetiology. NAAG was found to be elevated in 7 patients, but was normal in the majority, including patients with defined hypomyelinating disorders. CSF NAAG is not a universal marker of hypomyelination, and the mechanism of its elevation remains poorly understood.
Key words
N-acetylaspartylglutamate - hypomyelination - white matter disorder
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Correspondence
Nicole I. Wolf
Department of Child Neurology
VU University Medical Center
Postbox 7057
1007 MB Amsterdam
The Netherlands
Email: n.wolf@vumc.nl