EXCHANGE-TRANSFUSION IN ACUTE EPISODES OF MAPLE SYRUP URINE DISEASE - STUDIES ON BRANCHED-CHAIN AMINO AND KETO ACIDS

被引：12
|
作者：
WENDEL, U [1 ]
LANGENBECK, U [1 ]
LOMBECK, I [1 ]
BREMER, HJ [1 ]
机构：
[1] UNIV GOTTINGEN,INST HUMAN GENET,D-3400 GOTTINGEN,FED REP GER
来源：
EUROPEAN JOURNAL OF PEDIATRICS | 1982年 / 138卷 / 04期
关键词：
D O I：
10.1007/BF00442499
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
引用
收藏
页码：293 / 296
页数：4
相关论文
共 50 条
  • [41] MAPLE SYRUP URINE DISEASE - INHIBITION BY BRANCHED-CHAIN KETO-ACIDS OF ALPHA-KETOGLUTARATE DEHYDROGENASE IN DEVELOPING RAT AND HUMAN BRAIN
    PATEL, MS
    FEDERATION PROCEEDINGS, 1974, 33 (03) : 705 - 705
  • [42] Branched-chain α-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells
    Funchal, C
    dos Santos, AQ
    Jacques-Silva, MC
    Zamoner, A
    Gottfried, C
    Wajner, M
    Pessoa-Pureur, R
    METABOLIC BRAIN DISEASE, 2005, 20 (03) : 205 - 217
  • [43] Reduction of Glutamate Uptake into Cerebral Cortex of Developing Rats by the Branched-Chain Alpha-Keto Acids Accumulating in Maple Syrup Urine Disease
    Cláudia Funchal
    Aline Meyer Rosa
    Moacir Wajner
    Susana Wofchuk
    Regina Pessoa Pureur
    Neurochemical Research, 2004, 29 : 747 - 753
  • [44] Branched-Chain α-Keto Acids Accumulating in Maple Syrup Urine Disease Induce Reorganization of Phosphorylated GFAP in C6-Glioma Cells
    Cláudia Funchal
    André Quincozes dos Santos
    Maria Caroline Jacques-Silva
    Ariane Zamoner
    Carmem Gottfried
    Moacir Wajner
    Regina Pessoa-Pureur
    Metabolic Brain Disease, 2005, 20 : 205 - 217
  • [45] Effect of the branched-chain α-keto acids accumulating in maple syrup urine disease on S100B release from glial cells
    Funchal, Claudia
    Tramontina, Francine
    dos Santos, Andre Quincozes
    de Souza, Daniela Fraga
    Goncalves, Carlos Alberto
    Pessoa-Pureur, Regina
    Wajner, Moacir
    JOURNAL OF THE NEUROLOGICAL SCIENCES, 2007, 260 (1-2) : 87 - 94
  • [46] Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease
    Funchal, C
    Rosa, AM
    Wajner, M
    Wofchuk, S
    Pureur, RP
    NEUROCHEMICAL RESEARCH, 2004, 29 (04) : 747 - 753
  • [47] MAPLE SYRUP URINE DISEASE IN INDIAN BABY - BRANCHED CHAIN AMINO AND KETOACIDURIA
    DASTUR, DK
    MANGHANI, DK
    JOSHI, MK
    ADAVI, SV
    INDIAN JOURNAL OF MEDICAL RESEARCH, 1966, 54 (10) : 915 - &
  • [48] EVALUATION OF BRANCHED-CHAIN AMINO-ACID INTAKE IN CHILDREN WITH MAPLE SYRUP URINE DISEASE AND METHYLMALONIC ACIDURIA
    PARSONS, HG
    CARTER, RJ
    UNRATH, M
    SNYDER, FF
    JOURNAL OF INHERITED METABOLIC DISEASE, 1990, 13 (02) : 125 - 136
  • [49] A rapid LC-MS/MS assay for detection and monitoring of underivatized branched-chain amino acids in maple syrup urine disease
    Piri-Moghadam, Hamed
    Miller, Alan
    Pronger, Debra
    Vicente, Faye
    Charrow, Joel
    Haymond, Shannon
    Lin, David C.
    JOURNAL OF MASS SPECTROMETRY AND ADVANCES IN THE CLINICAL LAB, 2022, 24 : 107 - 117
  • [50] MAPLE SYRUP URINE DISEASE - BRANCHED-CHAIN AMINO-ACID CONCENTRATIONS AND METABOLISM IN CULTURED HUMAN LYMPHOBLASTS
    SKAPER, SD
    MOLDEN, DP
    SEEGMILLER, JE
    BIOCHEMICAL GENETICS, 1976, 14 (7-8) : 527 - 539
12345