Glucose-6-phosphatase dependent substrate transport in the glycogen storage disease type-1a mouse

被引:197
作者
Lei, KJ
Chen, HW
Pan, CJ
Ward, JM
Mosinger, B
Lee, EJ
Westphal, H
Mansfield, BC
Chou, JY
机构
[1] NICHHD, HERITABLE DISORDERS BRANCH, NIH, BETHESDA, MD 20892 USA
[2] NICHHD, LAB MAMMALIAN GENES & DEV, NIH, BETHESDA, MD 20892 USA
[3] NCI, VET & TUMOR PATHOL SECT, OFF LAB ANIM SCI, FREDERICK, MD 21702 USA
关键词
D O I
10.1038/ng0696-203
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Glycogen storage disease type 1a (GSD-1a) is caused by a deficiency in microsomal glucose-6-phosphatase (G6Pase), the key enzyme in glucose homeostasis. A G6Pase knockout mouse which mimics the pathophysiology of human GSD-1a patients was created to understand the pathogenesis of this disorder, to delineate the mechanisms of G6Pase catalysis, and to develop future therapeutic approaches. By examining G6Pase in the liver and kidney, the primary gluconeogenic tissues, we demonstrate that glucose-6-P transport and hydrolysis are performed by separate proteins which are tightly coupled. We propose a modified translocase catalytic unit model for G6Pase catalysis.
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收藏
页码:203 / 209
页数:7
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