Urinary 2-hydroxy-5-oxoproline, the lactam form of α-ketoglutaramate, is markedly increased in urea cycle disorders

Analytical and Bioanalytical Chemistry

Volumn 400, Issue 7, 2011, Pages 1843-1851

  Kuhara, Tomiko ^a   Inoue, Yoshito ^a   Ohse, Morimasa ^a   Krasnikov, Boris F ^b   Cooper, Arthur J L ^b  

^a KANAZAWA MEDICAL UNIVERSITY   (Japan)

^b NEW YORK MEDICAL COLLEGE   (United States)

Author keywords

Ketoglutaramate; 2 hydroxy 5 oxoproline; GC MS analysis of urine; Primary hyperammonemia; Secondary hyperammonemia; Urea cycle disorders

Indexed keywords

2-HYDROXY-5-OXOPROLINE; GAS CHROMATOGRAPHY/MASS SPECTROMETRY; GC/MS ANALYSIS OF URINE; GLUTAMINE LEVELS; HUMAN URINE; HYPERAMMONEMIA; KETO ACIDS; LOWER LIMIT OF QUANTIFICATIONS; METABOLIC DEFECTS; METHYLMALONIC ACIDEMIA; NITROGEN METABOLISM; PHYSIOLOGICAL CONDITION; PRIMARY HYPERAMMONEMIA; PROPIONIC; SECONDARY HYPERAMMONEMIA;

AMINO ACIDS; BODY FLUIDS; CHEMICAL ANALYSIS; DEFECTS; FATTY ACIDS; MASS SPECTROMETRY; PHYSIOLOGY; UREA;

METABOLISM;

2 OXOGLUTARIC ACID; ALPHA-KETOGLUTARIC ACID; AMMONIA; HYDROXYPROLINE; LACTAM;

ADULT; ARTICLE; BLOOD; CHILD; HUMAN; INFANT; MASS FRAGMENTOGRAPHY; METABOLISM; NEWBORN; PRESCHOOL CHILD; UREA CYCLE DISORDER; URINE;

ADULT; AMMONIA; CHILD; CHILD, PRESCHOOL; GAS CHROMATOGRAPHY-MASS SPECTROMETRY; HUMANS; HYDROXYPROLINE; INFANT; INFANT, NEWBORN; KETOGLUTARIC ACIDS; LACTAMS; UREA CYCLE DISORDERS, INBORN;

EID: 79956273527     PISSN: 16182642     EISSN: 16182650     Source Type: Journal    
DOI: 10.1007/s00216-011-4688-x     Document Type: Article

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