title: N-acetylaspartylglutamate in CNS hypomyelination.
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.
authors.affiliation: Department of Clinical Chemistry, Metabolic Unit, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: M M C
authors.initials: MM
authors.lastname: Wamelink
authors.forename: E
authors.initials: E
authors.lastname: Struys
authors.forename: U
authors.initials: U
authors.lastname: Holwerda
authors.forename: E A
authors.initials: EA
authors.lastname: Sistermans
authors.forename: R M L
authors.initials: RM
authors.lastname: van Spaendonk
authors.forename: D
authors.initials: D
authors.lastname: Halley
authors.forename: M A A P
authors.initials: MA
authors.lastname: Willemsen
authors.forename: C
authors.initials: C
authors.lastname: Jakobs
authors.forename: M S
authors.initials: MS
authors.lastname: van der Knaap
authors.forename: N I
authors.initials: NI
authors.lastname: Wolf
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003711:Demyelinating Diseases / Q000134:cerebrospinal fluid*; D004151:Dipeptides / Q000134:cerebrospinal fluid*; D005260:Female; D006801:Humans; D007223:Infant; D056784:Leukoencephalopathies / Q000134:cerebrospinal fluid*; D008279:Magnetic Resonance Imaging; D008297:Male; D014316:Tritium / Q000134:cerebrospinal fluid; D055815:Young Adult
chemical_list: D004151:Dipeptides; D014316:Tritium; C027172:isospaglumic acid