SUBCELLULAR-DISTRIBUTION AND COFACTOR FUNCTION OF HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE IN NORMAL AND MUTANT CULTURED SKIN FIBROBLASTS

被引:24
作者
DANNER, DJ [1 ]
ELSAS, LJ [1 ]
机构
[1] EMORY UNIV,SCH MED,DEPT PEDIAT,DIV MED GENET,ATLANTA,GA 30322
来源
BIOCHEMICAL MEDICINE | 1975年 / 13卷 / 01期
关键词
D O I
10.1016/0006-2944(75)90135-0
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
引用
收藏
页码:7 / 22
页数:16
相关论文
共 32 条
[1]  
BOWDEN JA, 1968, J BIOL CHEM, V243, P3526
[2]  
CONNELLY JL, 1968, J BIOL CHEM, V243, P1198
[3]   METABOLISM OF LEUCINE IN TISSUE CULTURE OF SKIN FIBROBLASTS OF MAPLE-SYRUP-URINE DISEASE [J].
DANCIS, J ;
LEVITZ, M ;
HUTZLER, J ;
JANSEN, V .
BIOCHIMICA ET BIOPHYSICA ACTA, 1963, 77 (03) :523-&
[4]   INTERMITTENT BRANCHED-CHAIN KETONURIA - VARIANT OF MAPLE-SYRUP-URINE DISEASE [J].
DANCIS, J ;
HUTZLER, J ;
ROKKONES, T .
NEW ENGLAND JOURNAL OF MEDICINE, 1967, 276 (02) :84-&
[5]   MAPLE SYRUP URINE DISEASE [J].
DANCIS, J ;
LEVITZ, ML ;
MILLER, S ;
WESTALL, RG .
BMJ-BRITISH MEDICAL JOURNAL, 1959, 1 (JAN10) :91-93
[6]  
DANCIS JOSEPH, 1967, P185
[7]  
DANNER DJ, 1965, THESIS U N DAKOTA
[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]   MAPLE SYRUP URINE DISEASE - COENZYME FUNCTION AND PRENATAL MONITORING [J].
ELSAS, LJ ;
PRIEST, JH ;
WHEELER, FB ;
DANNER, DJ ;
PASK, BA .
METABOLISM-CLINICAL AND EXPERIMENTAL, 1974, 23 (06) :569-579
[10]   MECHANISMS OF THIAMINE STIMULATED BRANCHED-CHAIN AMINO-ACID METABOLISM [J].
ELSAS, LJ ;
DANNER, DJ ;
ROGERS, BL ;
PRIEST, JH .
PEDIATRIC RESEARCH, 1974, 8 (04) :431-431