ASSESSMENT OF DEFICIENCIES OF FATTY ACYL-COA DEHYDROGENASES IN FIBROBLASTS, MUSCLE AND LIVER

被引:16
作者
SCHOLTE, HR
ROSS, JD
BLOM, W
BOONMAN, AMC
VANDIGGELEN, OP
HALL, CL
HUIJMANS, JGM
LUYTHOUWEN, IEM
KLEIJER, WJ
DEKLERK, JBC
PRZYREMBEL, H
VERDUIN, MHM
VERSTEGEN, JCM
机构
[1] ERASMUS UNIV,DEPT PEDIAT,3000 DR ROTTERDAM,NETHERLANDS
[2] ERASMUS UNIV,DEPT CLIN GENET,3000 DR ROTTERDAM,NETHERLANDS
[3] GEORGIA INST TECHNOL,SCH BIOL,ATLANTA,GA 30332
关键词
D O I
10.1007/BF02435973
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
[No abstract available]
引用
收藏
页码:347 / 352
页数:6
相关论文
共 12 条
[1]   SHORT-CHAIN ACYL-COENZYME-A DEHYDROGENASE-DEFICIENCY - CLINICAL AND BIOCHEMICAL-STUDIES IN 2 PATIENTS [J].
AMENDT, BA ;
GREENE, C ;
SWEETMAN, L ;
CLOHERTY, J ;
SHIH, V ;
MOON, A ;
TEEL, L ;
RHEAD, WJ .
JOURNAL OF CLINICAL INVESTIGATION, 1987, 79 (05) :1303-1309
[2]   THE LABORATORY DIAGNOSIS OF INBORN-ERRORS OF MITOCHONDRIAL FATTY-ACID OXIDATION [J].
BENNETT, MJ .
ANNALS OF CLINICAL BIOCHEMISTRY, 1990, 27 :519-531
[3]  
BENNETT MJ, 1990, FATTY ACID OXIDATION, P349
[4]   GENETIC DEFICIENCY OF SHORT-CHAIN ACYL-COENZYME-A DEHYDROGENASE IN CULTURED FIBROBLASTS FROM A PATIENT WITH MUSCLE CARNITINE DEFICIENCY AND SEVERE SKELETAL-MUSCLE WEAKNESS [J].
COATES, PM ;
HALE, DE ;
FINOCCHIARO, G ;
TANAKA, K ;
WINTER, SC .
JOURNAL OF CLINICAL INVESTIGATION, 1988, 81 (01) :171-175
[5]   SEPARATION, PROPERTIES, AND REGULATION OF ACYL COENZYME-A DEHYDROGENASES FROM BOVINE HEART AND LIVER [J].
DAVIDSON, B ;
SCHULZ, H .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1982, 213 (01) :155-162
[6]   FLUOROMETRIC ASSAY OF ACYL-COA DEHYDROGENASES IN NORMAL AND MUTANT HUMAN-FIBROBLASTS [J].
FRERMAN, FE ;
GOODMAN, SI .
BIOCHEMICAL MEDICINE, 1985, 33 (01) :38-44
[7]   AN ACYL-COENZYME A DEHYDROGENASE ASSAY UTILIZING THE FERRICENIUM ION [J].
LEHMAN, TC ;
HALE, DE ;
BHALA, A ;
THORPE, C .
ANALYTICAL BIOCHEMISTRY, 1990, 186 (02) :280-284
[8]  
Roe C, 1989, METABOLIC BASIS INHE, P889
[9]   DEFECTS IN OXIDATIVE-PHOSPHORYLATION - BIOCHEMICAL INVESTIGATIONS IN SKELETAL-MUSCLE AND EXPRESSION OF THE LESION IN OTHER CELLS [J].
SCHOLTE, HR ;
BUSCH, HFM ;
LUYTHOUWEN, IEM ;
VAANDRAGERVERDUIN, MHM ;
PRZYREMBEL, H ;
ARTS, WFM .
JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 :81-97
[10]  
TURNBULL DM, 1990, FATTY ACID OXIDATION, P313