BRANCHED-CHAIN ALPHA-KETO ACIDS ISOLATED AS OXIME DERIVATIVES - RELATIONSHIP TO CORRESPONDING HYDROXY-ACIDS AND AMINO-ACIDS IN MAPLE SYRUP URINE DISEASE

被引：50
作者：
LANCASTER, G
MAMER, OA
SCRIVER, CR
机构：
[1] ROY VICTORIA HOSP, DEPT MED, MASS SPECTR, MONTREAL, QUEBEC, CANADA
[2] MCGILL UNIV, MONTREAL CHILDRENS HOSP,RES INST,DEPT PEDIAT, DEBELLE LAB BIOCHEM GENET,2300 TUPPER, MONTREAL 108, QUEBEC, CANADA
来源：
METABOLISM-CLINICAL AND EXPERIMENTAL | 1974年 / 23卷 / 03期
关键词：
D O I：
10.1016/0026-0495(74)90064-X
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
引用
收藏
页码：257 / 265
页数：9
相关论文
共 22 条
[1]   INHIBITION OF PYRUVATE DECARBOXYLATION IN RAT BRAIN BY ALPHA-KETOISOCAPROIC ACID [J].
BOWDEN, JA ;
MCARTHUR, CL ;
FRIED, M .
BIOCHEMICAL MEDICINE, 1971, 5 (02) :101-&
[2]  
BOWDEN JA, 1968, J BIOL CHEM, V243, P3526
[3]   ALPHA-KETOISOCAPROIC ACID INHIBITION OF PYRUVATE AND ALPHA-KETOGLUTARATE OXIDATIVE DECARBOXYLATION IN RAT LIVER SLICES [J].
BOWDEN, JA ;
BRESTEL, EP ;
COPE, WT ;
MCARTHUR, CL ;
WESTFALL, DN ;
FRIED, M .
BIOCHEMICAL MEDICINE, 1970, 4 (01) :69-&
[4]   ENZYME-ACTIVITY IN CLASSICAL AND VARIANT FORMS OF MAPLE SYRUP URINE DISEASE [J].
DANCIS, J ;
COX, RP ;
SNYDERMAN, SE ;
HUTZLER, J .
JOURNAL OF PEDIATRICS, 1972, 81 (02) :312-+
[5]  
DANCIS J, 1972, METABOLIC BASIS INHE
[6]   STUDIES IN MAPLE SYRUP URINE DISEASE [J].
DENT, CE ;
WESTALL, RG .
ARCHIVES OF DISEASE IN CHILDHOOD, 1961, 36 (187) :259-+
[7]   FURTHER OBSERVATIONS ON BIOCHEMICAL LESION IN MAPLE SYRUP URINE DISEASE [J].
DREYFUS, PM ;
PRENSKY, AL .
NATURE, 1967, 214 (5085) :276-&
[8]   CLASSICAL MAPLE SYRUP URINE DISEASE - COFACTOR RESISTANCE [J].
ELSAS, LJ ;
WHEELER, FB ;
TRUSLER, S ;
PERL, DP ;
PASK, BA .
METABOLISM-CLINICAL AND EXPERIMENTAL, 1972, 21 (10) :929-&
[9]  
GOEDDE HW, 1967, AMINO ACID METABOLIS
[10]   GAS-CHROMATOGRAPHIC DIAGNOSIS OF INTERMITTENT MAPLE SYRUP URINE DISEASE (BRANCH-CHAIN KETOACIDURIA) [J].
GOMPERTZ, D ;
DRAFFAN, GH .
CLINICA CHIMICA ACTA, 1972, 40 (01) :5-&
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