Glucose–6–phosphatase dependent substrate transport in the glycogen storage disease type–1a mouse
KJ Lei, H Chen, CJ Pan, JM Ward, B Mosinger Jr Jr… - Nature …, 1996 - nature.com
KJ Lei, H Chen, CJ Pan, JM Ward, B Mosinger Jr Jr, EJ Lee, H Westphal, BC Mansfield…
Nature genetics, 1996•nature.comGlycogen 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–1 a 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 …
glucose–6–phosphatase (G6Pase), the key enzyme in glucose homeostasis. A G6Pase
knockout mouse which mimics the pathophysiology of human GSD–1 a 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 …
Abstract
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–1 a 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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