Article
Glucose 6-phosphate transport in fibroblast microsomes from glycogen storage disease type 1b patients: evidence for multiple glucose 6-phosphate transport systems.
The Biochemical journal - 15 Jul 2001
Leuzzi R, Fulceri R, Marcolongo P, Bánhegyi G, Zammarchi E, Stafford K, Burchell A, Benedetti A
Abstract excerpt
In liver endoplasmic reticulum the intralumenal glucose-6-phosphatase activity requires the operation of a glucose 6-phosphate transporter (G6PT1). Mutations in the gene encoding G6PT1 cause glycogen storage disease type 1b, which is characterized by a loss of glucose-6-phosphatase activity and impaired glucose homoeostasis. We describe a novel glucose 6-phosphate (G6P) transport activity in microsomes from human...
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