3-HYDROXY-3-METHYLGLUTARIC ACIDURIA - RESPONSE TO CARNITINE THERAPY AND FAT AND LEUCINE RESTRICTION

被引：27

作者：

DASOUKI, M

BUCHANAN, D

MERCER, N

GIBSON, KM

THOENE, J

机构：

[1] UNIV MICHIGAN,DEPT DIETET,NUTR SERV,ANN ARBOR,MI 48109

[2] UNIV CALIF SAN DIEGO,DEPT MICROBIOL IMMUNOL,DIV BIOCHEM GENET,LA JOLLA,CA 92093

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1987年 / 10卷 / 02期

关键词：

D O I：

10.1007/BF01800039

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

引用

页码：142 / 146

页数：5

共 18 条

[1]

BERRY HK, 1981, METABOLISM CLIN IMPL, P405

[2]

CHALMERS RA, 1984, J INHERIT METAB DIS, V7, P109

[3] URINARY-EXCRETION OF L-CARNITINE AND ACYLCARNITINES BY PATIENTS WITH DISORDERS OF ORGANIC-ACID METABOLISM - EVIDENCE FOR SECONDARY INSUFFICIENCY OF L-CARNITINE [J].

CHALMERS, RA ;

ROE, CR ;

STACEY, TE ;

HOPPEL, CL .

PEDIATRIC RESEARCH, 1984, 18 (12) :1325-1328

[4]

CLINKENBEARD KD, 1975, J BIOL CHEM, V250, P3108

[5] 3-HYDROXY-3-METHYLGLUTARYL COENZYME-A LYASE DEFICIENCY - POSTNATAL MANAGEMENT FOLLOWING PRENATAL DIAGNOSIS BY ANALYSIS OF MATERNAL URINE [J].

DURAN, M ;

SCHUTGENS, RBH ;

KETEL, A ;

HEYMANS, H ;

BERNTSSEN, MWJ ;

KETTING, D ;

WADMAN, SK .

JOURNAL OF PEDIATRICS, 1979, 95 (06) :1004-1007

[6]

FAULL K, 1976, NEW ENGL J MED, V294, P1013

[7] URINARY ORGANIC-ACID PROFILE ASSOCIATED WITH 3-HYDROXY-3-METHYLGLUTARIC ACIDURIA [J].

FAULL, KF ;

BOLTON, PD ;

HALPERN, B ;

HAMMOND, J ;

DANKS, DM .

CLINICA CHIMICA ACTA, 1976, 73 (03) :553-559

[8] 3-HYDROXY-3-METHYLGLUTARIC ACIDURIA - A NEW ASSAY OF 3-HYDROXY-3-METHYLGLUTARYL-COA LYASE USING HIGH-PERFORMANCE LIQUID-CHROMATOGRAPHY [J].

GIBSON, KM ;

SWEETMAN, L ;

NYHAN, WL ;

PAGE, TM ;

GREENE, C ;

CANN, HM .

CLINICA CHIMICA ACTA, 1982, 126 (02) :171-181

[9]

GOODMAN SI, 1981, DIAGNOSIS ORGANIC AC, P105

[10] 3-Hydroxy-3-Methylglutaric Aciduria [J].

Greene, Carol L. ;

Cann, Howard M. ;

Robinson, Brian H. ;

Gibson, Kenneth M. ;

Sweetman, Lawrence ;

Holm, Jan ;

Nyhan, William L. .

JOURNAL OF NEUROGENETICS, 1984, 1 (02) :165-173

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