DIRECT IDENTIFICATION OF PROPIONYLCARNITINE IN PROPIONIC ACIDEMIA - BIOCHEMICAL AND CLINICAL-RESULTS OF ORAL CARNITINE SUPPLEMENTATION

被引：15

作者：

DURAN, M ^{[1
]}

KETTING, D ^{[1
]}

BECKERINGH, TE ^{[1
]}

LEUPOLD, D ^{[1
]}

WADMAN, SK ^{[1
]}

机构：

[1] UNIV ULM,ZENTRUM KINDERHEILKUNDE,D-7900 ULM,FED REP GER

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1986年 / 9卷 / 02期

关键词：

D O I：

10.1007/BF01799460

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

引用

页码：202 / 207

页数：6

共 13 条

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[2] URINARY-EXCRETION OF L-CARNITINE AND ACYLCARNITINES BY PATIENTS WITH DISORDERS OF ORGANIC-ACID METABOLISM - EVIDENCE FOR SECONDARY INSUFFICIENCY OF L-CARNITINE
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[J]. PEDIATRIC RESEARCH, 1984, 18 (12) : 1325 - 1328
[3] HEPATIC KETOGENESIS AND MUSCLE CARNITINE DEFICIENCY
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[J]. NEUROLOGY, 1979, 29 (06) : 780 - 785
[4] PROPIONYLCARNITINE EXCRETION IN PROPIONIC AND METHYLMALONIC ACIDURIAS - A CAUSE OF CARNITINE DEFICIENCY
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[J]. CLINICA CHIMICA ACTA, 1984, 139 (01) : 13 - 21
[5] ISOVALERYLGLUCURONIDE, A NEW URINARY METABOLITE IN ISOVALERIC ACIDEMIA - IDENTIFICATION PROBLEMS DUE TO REARRANGEMENT REACTIONS
DORLAND, L
DURAN, M
WADMAN, SK
NIEDERWIESER, A
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KETTING, D
[J]. CLINICA CHIMICA ACTA, 1983, 134 (1-2) : 77 - 83
[6] VARIABILITY OF METABOLITE EXCRETION IN PROPIONICACIDEMIA
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[J]. CLINICA CHIMICA ACTA, 1978, 82 (1-2) : 93 - 99
[7] THE IDENTIFICATION OF ACYLCARNITINES BY DESORPTION CHEMICAL IONIZATION MASS-SPECTROMETRY
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KETTING, D
DORLAND, L
WADMAN, SK
[J]. JOURNAL OF INHERITED METABOLIC DISEASE, 1985, 8 : 143 - 144
[8] APPLICATION OF HIGH-RESOLUTION FAST ATOM BOMBARDMENT AND CONSTANT B-E RATIO LINKED SCANNING TO THE IDENTIFICATION AND ANALYSIS OF ACYLCARNITINES IN METABOLIC DISEASE
MILLINGTON, DS
ROE, CR
MALTBY, DA
[J]. BIOMEDICAL MASS SPECTROMETRY, 1984, 11 (05): : 236 - 241
[9] PEARSON DJ, 1974, METHOD ENZYMAT AN, V4, P1758
[10] L-CARNITINE ENHANCES EXCRETION OF PROPIONYL COENZYME-A AS PROPIONYLCARNITINE IN PROPIONIC ACIDEMIA
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MALTBY, DA
BOHAN, TP
HOPPEL, CL
[J]. JOURNAL OF CLINICAL INVESTIGATION, 1984, 73 (06) : 1785 - 1788

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