Probing into the function of the gene product responsible for glycogen storage disease type Ib

Abstract

This study aimed at directly assessing glucose 6-phosphate (G6P) transport by intact rat liver microsomes. Tracer uptake from labeled G6P occurred with T1/2 values that proved insensitive to unlabeled G6P or 100 μM vanadate, and could not be activated over background levels by intravesicular phosphate in the complete absence of G6P hydrolysis. [32P]Phosphate efflux was similarly unaffected by G6P or phosphate in the incubation medium. We conclude that the gene product responsible for glycogen storage disease type Ib is functionally distinct from the bacterial hexose phosphate transporter, which operates as an obligatory phosphate:phosphate or G6P:phosphate exchanger.