Impaired glucose transport in polymorphonuclear leukocytes in glycogen storage disease Ib.
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.
Bashan
N
N
Pediatric Research Laboratory, Soroka Medical Center, Beer Sheva, Israel.
Potashnik
R
R
Hagay
Y
Y
Moses
S W
SW
D017920:Antiporters; D001692:Biological Transport; D003837:Deoxy Sugars / Q000097:blood*; D003847:Deoxyglucose / Q000097:blood*; D006008:Glycogen Storage Disease / Q000097:blood*; D006801:Humans; D007700:Kinetics; D009004:Monosaccharide Transport Proteins; D009504:Neutrophils / Q000378:metabolism*; D010766:Phosphorylation; D010770:Phosphotransferases / Q000172:deficiency*
D017920:Antiporters; D003837:Deoxy Sugars; D009004:Monosaccharide Transport Proteins; C503531:SLC37A4 protein, human; C111261:glucose 6-phosphate(transporter); D003847:Deoxyglucose; D010770:Phosphotransferases