Impaired glucose transport in polymorphonuclear leukocytes in glycogen storage disease Ib

N. Bashan, R. Potashnik, Y. Hagay, S. W. Moses

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

A study of 2-deoxyglucose transport into polymorphonuclear leukocytes (PMN) was performed in three patients with glycogen storage disease (GSD) type Ib. The rate of 2-deoxyglucose transport into GSD Ib PMN was 30% of that of cells of normal controls. In GSD Ib lymphocytes, transport was normal. Km for 2-deoxyglucose in the PMN of one patient was within the normal range. The reduced transport was not due to the elevation in Km for 2-deoxyglucose nor to the decreased rate of phosphorylation of 2-deoxyglucose. The striking limitation of glucose transport across the cell membrane may account for the impairment of leukocyte function which is characteristic of GSD Ib.

Original languageEnglish
Pages (from-to)234-241
Number of pages8
JournalJournal of Inherited Metabolic Disease
Volume10
Issue number3
DOIs
StatePublished - 1 Sep 1987

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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