A direct evidence for defect in glucose-6-phosphate transport system in hepatic microsomal membrane of glycogen storage disease type IB.
Uptake of glucose-6-phosphate by microsomes of hepatocyte in rats, human controls and patients with glycogen storage disease type Ia and Ib was studied. In rat the uptake of glucose-6-phosphate increased rapidly and reached to a plateau, but mannose-6-phosphate was not accumulated. These findings indicate that a glucose-6-phosphate specific transport system exists in the microsomal membrane. In human controls and patients with glycogen storage disease type Ia the uptake of glucose-6-phosphate was clearly observed. On the other hand, no accumulation of it was detected in a patient with glycogen storage disease type Ib. These data provide a direct evidence of the defect in the glucose-6-phosphate transport system of hepatic microsomal membrane in glycogen storage disease type Ib.
D000818:Animals; D017920:Antiporters; D001692:Biological Transport; D005952:Glucose-6-Phosphatase / Q000378:metabolism; D019298:Glucose-6-Phosphate; D005958:Glucosephosphates / Q000378:metabolism; D005953:Glycogen Storage Disease Type I / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007425:Intracellular Membranes / Q000378:metabolism*; D007700:Kinetics; D008297:Male; D008862:Microsomes, Liver / Q000378:metabolism*; D009004:Monosaccharide Transport Proteins; D010770:Phosphotransferases / Q000378:metabolism*; D011755:Pyrophosphatases / Q000378:metabolism; D051381:Rats
D017920:Antiporters; D005958:Glucosephosphates; D009004:Monosaccharide Transport Proteins; C503531:SLC37A4 protein, human; C503533:Slc37a4 protein, rat; C111261:glucose 6-phosphate(transporter); D019298:Glucose-6-Phosphate; D010770:Phosphotransferases; D005952:Glucose-6-Phosphatase; D011755:Pyrophosphatases