Liver-specific glucose-6-phosphatase is not present in human placenta.

Published

Journal Article

Type I glycogen storage disease (McKusick 23220), an inherited absence or deficiency of glucose-6-phosphatase (EC 3.1.3.9) activity in the liver, kidney and intestine, is associated with the accumulation of glycogen in those organs. Previous reports have shown that glucose-6-phosphatase exists in human placenta and that detection of a heterozygote for this disorder from placenta might be possible. Our finding of a normal glucose-6-phosphatase activity in a placenta from a patient at risk for type Ia glycogen storage disease prompted us to examine in more detail placental glucose-6-phosphatase. Unexpectedly, we found the properties of the placental enzyme differed from that in normal liver, and the placental enzyme hydrolyzed glucose-6-phosphate, mannose-6-phosphate, beta-glycerol phosphate and glucose-1-phosphate equally well. Our data suggest the enzyme deficient in type I glycogen storage disease cannot be detected in placenta.

Full Text

Duke Authors

Cited Authors

  • Chen, YT; Kato, T

Published Date

  • 1985

Published In

Volume / Issue

  • 8 / 2

Start / End Page

  • 92 - 94

PubMed ID

  • 3023746

Pubmed Central ID

  • 3023746

International Standard Serial Number (ISSN)

  • 0141-8955

Digital Object Identifier (DOI)

  • 10.1007/bf01801675

Language

  • eng

Conference Location

  • United States