A direct evidence for defect in glucose-6-phosphate transport system in hepatic microsomal membrane of glycogen storage disease type IB |
| |
Authors: | Y Igarashi S Kato K Narisawa K Tada Y Amano T Mori S Takeuchi |
| |
Affiliation: | Department of Pediatrics, Tohoku University School of Medicine, Sendai 980, Japan;Department of Pediatrics, Shinshu University School of Medicine, Matsumoto 390, Japan |
| |
Abstract: | 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. |
| |
Keywords: | |
本文献已被 ScienceDirect 等数据库收录! |
|