Glucose 6-phosphate transport in fibroblast microsomes from glycogen storage disease type 1b patients: Evidence for multiple glucose 6-phosphate transport systems

R. Leuzzi, R. Fulceri, P. Marcolongo, G. Bánhegyi, E. Zammarchi, K. Stafford, A. Burchell, A. Benedetti

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21 Citations (Scopus)


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 fibroblasts and HeLa cells. This transport activity is unrelated to G6PT1 since: (i) it was similar in microsomes of skin fibroblasts from glycogen storage disease type 1b patients homozygous for mutations of the G6PT1 gene, and in microsomes from human control subjects; (ii) it was insensitive to the G6PT1 inhibitor chlorogenic acid; and (iii) it was equally active towards G6P and glucose 1-phosphate, whereas G6PT1 is highly selective for G6P. Taken together, our results provide evidence for the presence of multiple transporters for G6P (and other hexose phosphoesters) in the endoplasmic reticulum.

Original languageEnglish
Pages (from-to)557-562
Number of pages6
JournalBiochemical Journal
Issue number2
Publication statusPublished - Jul 15 2001



  • Endoplasmic reticulum
  • Glucose-6-phosphatase system
  • Glucose-6-phosphate translocase
  • Hexose phosphate transport system
  • Von Gierke's disease

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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