Minimal hepatic glucose-6-phosphatase-α activity required to sustain survival and prevent hepatocellular adenoma formation in murine glycogen storage disease type Ia

Author:

Lee Young Mok,Kim Goo-YoungORCID,Pan Chi-Jiunn,Mansfield Brian C.ORCID,Chou Janice Y.

Funder

Intramural Research Program of the Eunice Kennedy Shriver National Institute of Child Health and Human Development

National Institutes of Health

The Children's Fund for Glycogen Storage Disease Research

Publisher

Elsevier BV

Subject

Endocrinology,Genetics,Molecular Biology

Reference18 articles.

1. Type I glycogen storage diseases: disorders of the glucose-6-phosphatase complex;Chou;Curr. Mol. Med.,2002

2. Glycogen storage disease type I and G6Pase-β deficiency: etiology and therapy;Chou;Nat. Rev. Endocrinol.,2010

3. Transmembrane topology of glucose-6-phosphatase;Pan;J. Biol. Chem.,1998

4. Quantitative aspects of relationship between glucose 6-phosphate transport and hydrolysis for liver microsomal glucose-6-phosphatase system. Selective thermal inactivation of catalytic component in situ at acid pH;Arion;J. Biol. Chem.,1976

5. Continuous nocturnal intragastric feeding for management of type 1 glycogen-storage disease;Greene;N. Engl. J. Med.,1976

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