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1 H‐NMR Studies of Urine in Propionic Acidemia and Methylmalonic Acidemia
Author(s) -
Kodama Soichi,
Sugiura Makiko,
Nakao Hideto,
Kobayashi Ken,
Miyoshi Mari,
Yoshii Katsuhiko,
Komatsu Mikio,
Sakurai Takashi
Publication year - 1991
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1991.tb01534.x
Subject(s) - methylmalonic acidemia , propionic acidemia , medicine , urine , glycine , chromatography , amino acid , biochemistry , chemistry
The present study deals with the usefulness of 1 H‐NMR spectroscopy in the chemical diagnosis of propionic acidemia and methylmalonic acidemia. A Varian VXR‐500 spectrometer was used. x H‐NMR spectra of urine from a patient with propionic acidemia show peaks of 3‐hydroxy‐n‐valerate, methylcitrate, propionyl glycine, 3‐hydroxy‐n‐butyrate, lactate, 3‐hydroxypropionate, tiglylglycine and hippurate. The assignment was made possible by the use of 2D COSY. The spectra from a patient with methylmalonic acidemia show methylmalonate and glycine. 1 H‐NMR spectroscopy has the advantages of requiring a short time for analysis, relatively small amounts of urine and no preliminary extraction.