PERITONEAL-DIALYSIS IN MAPLE-SYRUP-URINE DISEASE - STUDIES ON BRANCHED-CHAIN AMINO AND KETO ACIDS

被引：23
|
作者：
WENDEL, U [1 ]
BECKER, K [1 ]
PRZYREMBEL, H [1 ]
BULLA, M [1 ]
MANEGOLD, C [1 ]
MENCHHOINOWSKI, A [1 ]
LANGENBECK, U [1 ]
机构：
[1] UNIV COLOGNE,CHILDRENS HOSP,D-5000 COLOGNE 41,FED REP GER
来源：
EUROPEAN JOURNAL OF PEDIATRICS | 1980年 / 134卷 / 01期
关键词：
D O I：
10.1007/BF00442404
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
引用
收藏
页码：57 / 63
页数：7
相关论文
共 50 条
  • [41] BRANCHED-CHAIN AMINO ACID-FREE HYPERALIMENTATION THERAPY OF KETOACIDOSIS IN MAPLE SYRUP URINE DISEASE
    BERRY, GT
    KAPLAN, P
    LEVINE, F
    HEIDENREICH, RA
    MAZUR, AT
    YUDKOFF, M
    SEGAL, S
    PEDIATRIC RESEARCH, 1989, 25 (04) : A195 - A195
  • [42] Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease
    Thais C. Vilela
    Giselli Scaini
    Camila B. Furlanetto
    Matheus A. B. Pasquali
    João Paulo A. Santos
    Daniel P. Gelain
    José Cláudio F. Moreira
    Patrícia F. Schuck
    Gustavo C. Ferreira
    Emilio L. Streck
    Metabolic Brain Disease, 2017, 32 : 115 - 122
  • [43] Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease
    Vilela, Thais C.
    Scaini, Giselli
    Furlanetto, Camila B.
    Pasquali, Matheus A. B.
    Santos, Joao Paulo A.
    Gelain, Daniel P.
    Moreira, Jose Claudio F.
    Schuck, Patricia F.
    Ferreira, Gustavo C.
    Streck, Emilio L.
    METABOLIC BRAIN DISEASE, 2017, 32 (01) : 115 - 122
  • [44] FUNCTIONAL DIFFERENCES IN THE CATABOLISM OF BRANCHED-CHAIN L-AMINO-ACIDS IN CULTURED NORMAL AND MAPLE SYRUP URINE DISEASE FIBROBLASTS
    SCHADEWALDT, P
    WENDEL, U
    BIOCHEMICAL MEDICINE AND METABOLIC BIOLOGY, 1989, 41 (02): : 105 - 116
  • [45] 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
  • [46] HYPERALIMENTATION AND PERITONEAL-DIALYSIS DURING ACUTE METABOLIC DECOMPENSATION IN MAPLE SYRUP URINE DISEASE
    SHIH, VE
    HERRIN, JT
    ERICKSON, AM
    PEDIATRIC RESEARCH, 1975, 9 (04) : 355 - 355
  • [47] 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
  • [48] 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
  • [49] 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
  • [50] 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
12345