The ontogeny of human hepatic microsomal glucose-6-phosphatase proteins.
We have studied 250 human liver biopsy samples to determine the ontogeny of the microsomal glucose-6-phosphatase (EC 3.1.3.9) system. Human hepatic glucose-6-phosphatase enzyme activity develops at 11 weeks' gestation and slowly increases to approximately 10% of adult activity at term. In the first week after birth, activity rises to adult values. Increases in enzyme activity coincide with increasing concentrations of the glucose-6-phosphatase enzyme protein. The phosphate/pyrophosphate transport protein (T2) of the human hepatic glucose-6-phosphatase complex develops at a different rate from that of the enzyme. Our study shows that the development of rat and human glucose-6-phosphatase activities are completely different. We conclude that deficiencies of the proteins in the microsomal glucose-6-phosphatase complex can be diagnosed with much more certainty perinatally than prenatally.
Burchell
A
A
Centre for Perinatal and Paediatric Research, Ninewells Hospital, University of Dundee, U.K.
Gibb
L
L
Waddell
I D
ID
Giles
M
M
Hume
R
R
D000328:Adult; D004790:Enzyme Induction / Q000502:physiology; D005333:Fetus / Q000201:enzymology* / Q000378:metabolism; D005865:Gestational Age; D005952:Glucose-6-Phosphatase / Q000096:biosynthesis*; D005953:Glycogen Storage Disease Type I / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D008862:Microsomes, Liver / Q000201:enzymology* / Q000378:metabolism; D011506:Proteins / Q000378:metabolism
D011506:Proteins; D005952:Glucose-6-Phosphatase