ENZYMOLOGIC STUDIES AND THERAPY OF LEIGHS DISEASE ASSOCIATED WITH PYRUVATE DECARBOXYLASE DEFICIENCY

被引:39
作者
TOSHIMA, K
KURODA, Y
HASHIMOTO, T
ITO, M
WATANABE, T
MIYAO, M
II, K
机构
[1] UNIV TOKUSHIMA,SCH MED,DEPT PEDIAT,TOKUSHIMA 770,JAPAN
[2] UNIV TOKUSHIMA,SCH MED,DEPT PATHOL 1,TOKUSHIMA 770,JAPAN
关键词
D O I
10.1203/00006450-198206000-00006
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:430 / 435
页数:6
相关论文
共 41 条
[1]   PYRUVATE-CARBOXYLASE DEFICIENCY AND LACTIC-ACIDOSIS IN A RETARDED-CHILD WITHOUT LEIGHS DISEASE [J].
ATKIN, BM ;
BUIST, NRM ;
UTTER, MF ;
LEITER, AB ;
BANKER, BQ .
PEDIATRIC RESEARCH, 1979, 13 (02) :109-116
[2]   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-+
[3]  
BLASS J P, 1970, Journal of Clinical Investigation, V49, P423, DOI 10.1172/JCI106251
[4]  
BLASS JP, 1976, LANCET, V1, P1237
[5]   RAPID DIAGNOSIS OF PYRUVATE AND KETOGLUTARATE DEHYDROGENASE DEFICIENCIES IN PLATELET-ENRICHED PREPARATIONS FROM BLOOD [J].
BLASS, JP ;
CEDERBAUM, SD ;
KARK, RAP .
CLINICA CHIMICA ACTA, 1977, 75 (01) :21-30
[6]   LOW ACTIVITIES OF PYRUVATE AND OXOGLUTARATE DEHYDROGENASE COMPLEXES IN 5 PATIENTS WITH FRIEDREICHS ATAXIA [J].
BLASS, JP ;
KARK, RAP ;
MENON, NK .
NEW ENGLAND JOURNAL OF MEDICINE, 1976, 295 (02) :62-67
[7]   KINETIC PROPERTIES OF PARTIALLY PURIFIED PYRUVATE DEHYDROGENASE COMPLEX OF OX BRAIN [J].
BLASS, JP ;
LEWIS, CA .
BIOCHEMICAL JOURNAL, 1973, 131 (01) :31-37
[8]   MUSCLE FIBER TYPES - HOW MANY AND WHAT KIND [J].
BROOKE, MH ;
KAISER, KK .
ARCHIVES OF NEUROLOGY, 1970, 23 (04) :369-&
[9]   SENSITIVITY TO CARBOHYDRATE IN A PATIENT WITH FAMILIAL INTERMITTENT LACTIC-ACIDOSIS AND PYRUVATE-DEHYDROGENASE DEFICIENCY [J].
CEDERBAUM, SD ;
BLASS, JP ;
MINKOFF, N ;
BROWN, WJ ;
COTTON, ME ;
HARRIS, SH .
PEDIATRIC RESEARCH, 1976, 10 (08) :713-720
[10]   EXPERIENCE WITH PHOSPHORYL TRANSFERASE INHIBITION IN SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY [J].
COOPER, JR ;
PINCUS, JH ;
ITOKAWA, Y ;
PIROS, K .
NEW ENGLAND JOURNAL OF MEDICINE, 1970, 283 (15) :793-&