MUTATION OF THE E1-ALPHA SUBUNIT OF THE PYRUVATE-DEHYDROGENASE COMPLEX, IN RELATION TO HETEROGENEITY

被引:11
作者
KITANO, A
ENDO, F
MATSUDA, I
MIYABAYASHI, S
DAHL, HHM
机构
[1] KUMAMOTO UNIV,SCH MED,DEPT PEDIAT,KUMAMOTO 860,JAPAN
[2] UNIV MELBOURNE,ROYAL CHILDRENS HOSP,MURDOCH INST RES BIRTH DEFECTS,PARKVILLE,VIC 3052,AUSTRALIA
[3] TOHOKU UNIV,SCH MED,DEPT PEDIAT,SENDAI,MIYAGI 980,JAPAN
关键词
D O I
10.1007/BF01800710
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
引用
收藏
页码:97 / 107
页数:11
相关论文
共 26 条
[1]   ALPHA-KETO ACID DEHYDROGENASE COMPLEXES .16. STUDIES ON SUBUNIT STRUCTURE OF PYRUVATE DEHYDROGENASE COMPLEXES FROM BOVINE KIDNEY AND HEART [J].
BARRERA, CR ;
REED, LJ ;
LINN, TC ;
MUNK, P ;
NAMIHIRA, G ;
ELEY, MH ;
HAMILTON, L .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1972, 148 (02) :343-+
[2]  
BLASS JP, 1983, METABOLIC BASIS INHE, P193
[3]   HYPERAMMONEMIA AND LACTIC-ACIDOSIS IN A PATIENT WITH PYRUVATE-DEHYDROGENASE DEFICIENCY [J].
BROWN, GK ;
SCHOLEM, RD ;
HUNT, SM ;
HARRISON, JR ;
POLLARD, AC .
JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 (04) :359-366
[4]  
CHOMCZYNSKI P, 1987, ANAL BIOCHEM, V162, P156, DOI 10.1016/0003-2697(87)90021-2
[5]  
DAHL HHM, 1987, J BIOL CHEM, V262, P7398
[6]   COMPONENT-X - AN IMMUNOLOGICALLY DISTINCT POLYPEPTIDE ASSOCIATED WITH MAMMALIAN PYRUVATE-DEHYDROGENASE MULTI-ENZYME COMPLEX [J].
DEMARCUCCI, O ;
LINDSAY, JG .
EUROPEAN JOURNAL OF BIOCHEMISTRY, 1985, 149 (03) :641-648
[7]   LOW IMMUNOGENICITY OF THE COMMON LIPOAMIDE DEHYDROGENASE SUBUNIT (E3) OF MAMMALIAN PYRUVATE-DEHYDROGENASE AND 2-OXOGLUTARATE DEHYDROGENASE MULTIENZYME COMPLEXES [J].
DEMARCUCCI, OL ;
HUNTER, A ;
LINDSAY, JG .
BIOCHEMICAL JOURNAL, 1985, 226 (02) :509-517
[8]  
DEMEIRLEIR L, 1988, J BIOL CHEM, V263, P1991
[9]   A TECHNIQUE FOR RADIOLABELING DNA RESTRICTION ENDONUCLEASE FRAGMENTS TO HIGH SPECIFIC ACTIVITY [J].
FEINBERG, AP ;
VOGELSTEIN, B .
ANALYTICAL BIOCHEMISTRY, 1983, 132 (01) :6-13
[10]   PURIFICATION AND PROPERTIES OF PIGEON BREAST MUSCLE ALPHA-KETO ACID DEHYDROGENASE COMPLEXES [J].
FURUTA, S ;
SHINDO, Y ;
HASHIMOTO, T .
JOURNAL OF BIOCHEMISTRY, 1977, 81 (06) :1839-1847