QUANTITATION OF URINARY CARNITINE ESTERS IN A PATIENT WITH MEDIUM-CHAIN ACYL COENZYME-A DEHYDROGENASE-DEFICIENCY - EFFECT OF METABOLIC STATE AND L-CARNITINE THERAPY

被引:27
作者
SCHMIDTSOMMERFELD, E
PENN, D
KERNER, J
BIEBER, LL
ROSSI, TM
LEBENTHAL, E
机构
[1] MICHIGAN STATE UNIV, DEPT BIOCHEM, E LANSING, MI 48824 USA
[2] SUNY BUFFALO, DEPT PEDIAT, DIV GASTROENTEROL & NUTR, BUFFALO, NY 14260 USA
关键词
D O I
10.1016/S0022-3476(89)80284-7
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:577 / 582
页数:6
相关论文
共 24 条
  • [1] ACYLCOENZYME-A DEHYDROGENASE-DEFICIENCY IN HEART-TISSUE FROM INFANTS WHO DIED UNEXPECTEDLY WITH FATTY CHANGE IN THE LIVER
    ALLISON, F
    BENNETT, MJ
    VARIEND, S
    ENGEL, PC
    [J]. BRITISH MEDICAL JOURNAL, 1988, 296 (6614) : 11 - 12
  • [2] BIEBER LL, 1986, METHOD ENZYMOL, V123, P264, DOI 10.1016/S0076-6879(86)23030-X
  • [3] PROPIONYLCARNITINE PHYSIOLOGICAL VARIATIONS IN VIVO
    BOHMER, T
    BREMER, J
    [J]. BIOCHIMICA ET BIOPHYSICA ACTA, 1968, 152 (03) : 559 - &
  • [4] GENETIC DEFICIENCY OF MEDIUM-CHAIN ACYL COENZYME-A DEHYDROGENASE - STUDIES IN CULTURED SKIN FIBROBLASTS AND PERIPHERAL MONONUCLEAR LEUKOCYTES
    COATES, PM
    HALE, DE
    STANLEY, CA
    CORKEY, BE
    CORTNER, JA
    [J]. PEDIATRIC RESEARCH, 1985, 19 (07) : 671 - 676
  • [5] OCTANOIC ACIDEMIA AND OCTANOYLCARNITINE EXCRETION WITH DICARBOXYLIC ACIDURIA DUE TO DEFECTIVE OXIDATION OF MEDIUM-CHAIN FATTY-ACIDS
    DURAN, M
    MITCHELL, G
    DEKLERK, JBC
    DEJAGER, JP
    HOFKAMP, M
    BRUINVIS, L
    KETTING, D
    SAUDUBRAY, JM
    WADMAN, SK
    [J]. JOURNAL OF PEDIATRICS, 1985, 107 (03) : 397 - 404
  • [6] CHEMICAL DIAGNOSIS OF INHERITED DEFECTS OF FATTY-ACID METABOLISM AND KETOGENESIS
    DURAN, M
    WADMAN, SK
    [J]. ENZYME, 1987, 38 (1-4) : 115 - 123
  • [7] PRIMARY SYSTEMIC CARNITINE DEFICIENCY .2. RENAL HANDLING OF CARNITINE
    ENGEL, AG
    REBOUCHE, CJ
    WILSON, DM
    GLASGOW, AM
    ROMSHE, CA
    CRUSE, RP
    [J]. NEUROLOGY, 1981, 31 (07) : 819 - 825
  • [8] DEFICIENCY OF MEDIUM CHAIN FATTY ACYLCOENZYME-A DEHYDROGENASE PRESENTING AS THE SUDDEN INFANT DEATH SYNDROME
    HOWAT, AJ
    BENNETT, MJ
    VARIEND, S
    SHAW, L
    [J]. BRITISH MEDICAL JOURNAL, 1984, 288 (6422) : 976 - 976
  • [9] A RADIOISOTOPIC-EXCHANGE METHOD FOR QUANTITATION OF SHORT-CHAIN (ACID-SOLUBLE) ACYLCARNITINES
    KERNER, J
    BIEBER, LL
    [J]. ANALYTICAL BIOCHEMISTRY, 1983, 134 (02) : 459 - 466
  • [10] KERNER J, 1985, PREP BIOCHEM, V15, P237