A SYNOPSIS OF THE UNCONJUGATED ACIDIC TRANSAMINATION METABOLITES OF PHENYLALANINE IN PHENYLKETONURIA

被引:13
作者
LANGENBECK, U
BEHBEHANI, A
MENCHHOINOWSKI, A
机构
[1] UNIV FRANKFURT KLINIKUM,INST HUMAN GENET,W-6000 FRANKFURT 70,GERMANY
[2] UNIV GOTTINGEN,CHILDRENS HOSP,W-3400 GOTTINGEN,GERMANY
关键词
D O I
10.1007/BF01800355
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
We present blood and urine levels of unconjugated o-hydroxyphenylacetic, phenyllactic and phenylpyruvic acids in 61 children (2 years of age and above) and juveniles with phenylketonuria on or partially off diet. The samples were obtained during 185 scheduled outpatient visits and have been analysed with gas chromatographic methods. The compiled data define reference ranges of phenylalanine transamination capacity and of renal transport of metabolites which may be of value in further studies on the pathogenesis of phenylketonuria.
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页码:136 / 144
页数:9
相关论文
共 39 条
  • [11] MOLECULAR-GENETICS OF PKU
    GUTTLER, F
    WOO, SLC
    [J]. JOURNAL OF INHERITED METABOLIC DISEASE, 1986, 9 : 58 - 68
  • [12] ENZYMATIC DETERMINATION OF L-PHENYLALANINE AND PHENYLPYRUVATE WITH L-PHENYLALANINE DEHYDROGENASE
    HUMMEL, W
    SCHUTTE, H
    KULA, MR
    [J]. ANALYTICAL BIOCHEMISTRY, 1988, 170 (02) : 397 - 401
  • [13] KAUFMAN S, 1990, J PEDIATR-US, V116, P665, DOI 10.1016/S0022-3476(05)81625-7
  • [14] AN EVALUATION OF THE POSSIBLE NEUROTOXICITY OF METABOLITES OF PHENYLALANINE
    KAUFMAN, S
    [J]. JOURNAL OF PEDIATRICS, 1989, 114 (05) : 895 - 900
  • [15] KITAGAWA T, 1975, CLIN CHEM, V21, P735
  • [16] KITAGAWA T, 1974, CLIN CHEM, V20, P1543
  • [17] BIOCHEMICAL AND NEUROPSYCHOLOGICAL EFFECTS OF ELEVATED PLASMA PHENYLALANINE IN PATIENTS WITH TREATED PHENYLKETONURIA - A MODEL FOR THE STUDY OF PHENYLALANINE AND BRAIN-FUNCTION IN MAN
    KRAUSE, W
    HALMINSKI, M
    MCDONALD, L
    DEMBURE, P
    SALVO, R
    FREIDES, D
    ELSAS, L
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1985, 75 (01) : 40 - 48
  • [18] RENAL TRANSPORT OF AROMATIC-ACIDS IN PATIENTS WITH PHENYLKETONURIA
    LANGENBECK, U
    BEHBEHANI, A
    LUTHE, H
    [J]. JOURNAL OF INHERITED METABOLIC DISEASE, 1981, 4 (02) : 69 - 70
  • [19] O-TRIMETHYLSILYLQUINOXALINOL DERIVATIVES OF AROMATIC ALPHA-KETO ACIDS - MASS-SPECTRA AND QUANTITATIVE GAS-CHROMATOGRAPHY
    LANGENBECK, U
    MENCHHOINOWSKI, A
    DIECKMANN, KP
    MOHRING, HU
    PETERSEN, M
    [J]. JOURNAL OF CHROMATOGRAPHY, 1978, 145 (02): : 185 - 193
  • [20] ABSENCE OF A SIGNIFICANT RENAL THRESHOLD FOR 2 AROMATIC-ACIDS IN PHENYLKETONURIC CHILDREN OVER 2 YEARS OF AGE
    LANGENBECK, U
    BEHBEHANI, A
    MENCHHOINOWSKI, A
    PETERSEN, M
    [J]. EUROPEAN JOURNAL OF PEDIATRICS, 1980, 134 (02) : 115 - 118