G6PC hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (liver)

Stable Identifier
R-HSA-71825
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC - liver)
Locations in the PathwayBrowser
Summation

Glucose-6-phosphatase (G6PC) associated with the inner face of the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate to glucose and orthophosphate. This reaction is essentially irreversible (Lei et al. 1993, Ghosh et al. 2002). Defects in glucose-6-phosphatase are the cause of glycogen storage disease type 1a (Lei et al. 1993, 1995, Chou and Mansfield 2008).

Literature References
PubMed ID Title Journal Year
12093795 The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis

Ghosh, A, Shieh, JJ, Pan, CJ, Sun, MS, Chou, JY

J. Biol. Chem. 2002
7573034 Genetic basis of glycogen storage disease type 1a: Prevalent mutations at the glucose-6-phosphatase locus

Lei, K-J, Chen, Y-T, Chen, H, Wong, L-JC, Liu, J-L, McConkie-Rosell, A, Van Hove, JLK, Ou, HC-Y, Yeh, NJ, Pan, LY, Chou, JY

Am J Hum Genet 1995
18449899 Mutations in the glucose-6-phosphatase-alpha (G6PC) gene that cause type Ia glycogen storage disease

Chou, JY, Mansfield, BC

Hum. Mutat. 2008
8211187 Mutations in the glucose-6-phosphatase gene that cause glycogen storage disease type 1a

Lei, K-J, Shelly, LL, Pan, CJ, Sidbury, JB, Chou, JY

Science 1993
Participants
Participant Of
hasEvent
Catalyst Activity
Catalyst Activity
Title
glucose-6-phosphatase activity of G6PC [endoplasmic reticulum membrane]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Reviewed