TYPE-IB GLYCOGEN-STORAGE DISEASE IS CAUSED BY A DEFECT IN THE GLUCOSE-6-PHOSPHATE TRANSLOCASE OF THE MICROSOMAL GLUCOSE-6-PHOSPHATASE SYSTEM

被引:0
|
作者
LANGE, AJ
ARION, WJ
BEAUDET, AL
机构
[1] CORNELL UNIV,NEW YORK STATE COLL VET MED,DEPT PHYSIOL BIOCHEM & PHARMACOL,ITHACA,NY 14853
[2] BAYLOR COLL MED,DEPT PEDIAT,HOUSTON,TX 77030
[3] HOWARD HUGHES MED INST LAB,MIAMI,FL
关键词
D O I
暂无
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
引用
收藏
页码:8381 / 8384
页数:4
相关论文
共 50 条
  • [21] Three thiol groups are important for the activity of the liver microsomal glucose-6-phosphatase system - Unusual behavior of one thiol located in the glucose-6-phosphate translocase
    Clottes, E
    Burchell, A
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1998, 273 (31) : 19391 - 19397
  • [22] α-Bromophosphonate analogs of glucose-6-phosphate are inhibitors of glucose-6-phosphatase
    Downey, A. Michael
    Cairo, Christopher W.
    CARBOHYDRATE RESEARCH, 2013, 381 : 123 - 132
  • [23] A gene on chromosome 11q23 coding for a putative glucose-6-phosphate translocase is mutated in glycogen-storage disease types Ib and Ic
    Veiga-da-Cunha, M
    Gerin, I
    Chen, YT
    de Barsy, T
    de Lonlay, P
    Dionisi-Vici, C
    Fenske, CD
    Lee, PJ
    Leonard, JV
    Maire, I
    McConkie-Rosell, A
    Schweitzer, S
    Vikkula, M
    Van Schaftingen, E
    AMERICAN JOURNAL OF HUMAN GENETICS, 1998, 63 (04) : 976 - 983
  • [24] INVIVO EVIDENCE FOR DEFECTIVE ACTIVITY OF GLUCOSE-6-PHOSPHATASE IN TYPE-IB GLYCOGENOSIS
    SANN, L
    MATHIEU, M
    BOURGEOIS, J
    BIENVENU, J
    BETHENOD, M
    JOURNAL OF PEDIATRICS, 1980, 96 (04): : 691 - 694
  • [25] GLUCOSE-6-PHOSPHATE TRANSPORT AND MICROSOMAL GLUCOSE-6-PHOSPHATASE .3. QUANTITATIVE ASPECTS OF RELATIONSHIP BETWEEN GLUCOSE-6-PHOSPHATE TRANSPORT AND HYDROLYSIS FOR LIVER MICROSOMAL GLUCOSE-6-PHOSPHATASE SYSTEM - SELECTIVE THERMAL INACTIVATION OF CATALYTIC COMPONENT INSITU AT ACID PH
    ARION, WJ
    LANGE, AJ
    BALLAS, LM
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1976, 251 (21) : 6784 - 6790
  • [26] Functional analysis of mutations in the glucose-6-phosphate transporter that cause glycogen storage disease type Ib
    Chen, Shih-Yin
    Pan, Chi-Jiunn
    Lee, Soojung
    Peng, Wentao
    Chou, Janice Y.
    MOLECULAR GENETICS AND METABOLISM, 2008, 95 (04) : 220 - 223
  • [27] Glycogen storage disease type Ib: role of glucose-6-phosphate transporter in cell metabolism and function
    Sim, Sang Wan
    Weinstein, David A.
    Lee, Young Mok
    Jun, Hyun Sik
    FEBS LETTERS, 2020, 594 (01) : 3 - 18
  • [28] Ontogeny of the catalytic subunit and putative glucose-6-phosphate transporter proteins of the rat microsomal liver glucose-6-phosphatase system
    Méchin, MC
    Annabi, B
    Pegorier, JP
    van de Werve, G
    METABOLISM-CLINICAL AND EXPERIMENTAL, 2000, 49 (09): : 1200 - 1203
  • [29] ASTROCYTIC GLUCOSE-6-PHOSPHATASE AND THE PERMEABILITY OF BRAIN MICROSOMES TO GLUCOSE-6-PHOSPHATE
    FORSYTH, RJ
    BARTLETT, K
    BURCHELL, A
    SCOTT, HM
    EYRE, JA
    BIOCHEMICAL JOURNAL, 1993, 294 : 145 - 151
  • [30] Glucose-6-Phosphatase and Glucose-6-Phosphate Dehydrogenase Deficiency: How Are They Different?
    Rubarth, Lori Baas
    NEONATAL NETWORK, 2012, 31 (01): : 45 - 47