caa a22 4500 469043911 CHVBK 20180323132818.0 cr unu---uuuuu 170328e19920101xx s 000 0 eng 10.1007/BF01800351 doi (NATIONALLICENCE)springer-10.1007/BF01800351 Quantification of N -acetyl-l-aspartic acid in urine by isotope dilution gas chromatography-mass spectrometry [Elektronische Daten] [R. Kelley, J. Stamas] Summary: A method for quantification ofN-acetyl-l-aspartic acid by isotope dilution gas chromatography-mass spectrometry using15 N-[2H3]acetyl-l-aspartic acid is described. The method is sufficiently sensitive to be used with solvent extraction techniques commonly employed for urinary organic acid analysis. The mean concentration ofN-acetyl-l-aspartic acid in 80 normal and abnormal control urine specimens was 19.7±10.8 mg/g creatinine (12.7±7.8 mmol/mol creatinine). Seven patients, ages 9 months to 7 years, with Canavan-van Bogaert syndrome had urinaryN-acetyl-l-aspartic acid levels from 606 to 4760 mg/g creatinine. The method can also be used with cerebrospinal fluid, in which the concentration ofN-acetyl-l-aspartic acid is about one-tenth of that in urine. SSIEM and Kluwer Academic Publishers, 1992 Kelley R. Department of Pediatrics, Johns Hopkins University School of Medicine, 701 North Broadway, 21205, Baltimore, MD, USA aut Stamas J. Department of Pediatrics, Johns Hopkins University School of Medicine, 701 North Broadway, 21205, Baltimore, MD, USA aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 97-104 0141-8955 15:1<97 1992 15 10545 https://doi.org/10.1007/BF01800351 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800351 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kelley R. Department of Pediatrics, Johns Hopkins University School of Medicine, 701 North Broadway, 21205, Baltimore, MD, USA aut NATIONALLICENCE 700 1- Stamas J. Department of Pediatrics, Johns Hopkins University School of Medicine, 701 North Broadway, 21205, Baltimore, MD, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 97-104 0141-8955 15:1<97 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer