G6PC hydrolyzes G6P to Glc and Pi (liver)

Stable Identifier
Reaction [transition]
Homo sapiens
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC - liver)
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Glucose-6-phosphatase (G6PC) associated with the inner face of the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate (G6P) to glucose (Glc) and orthophosphate (Pi). This reaction is essentially irreversible (Lei et al. 1993, Ghosh et al. 2002). Defects in G6PC 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
7573034 Genetic basis of glycogen storage disease type 1a: Prevalent mutations at the glucose-6-phosphatase locus

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

Am J Hum Genet 1995
12093795 The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis

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

J. Biol. Chem. 2002
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

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

Science 1993
Catalyst Activity

glucose-6-phosphatase activity of G6PC [endoplasmic reticulum membrane]

Orthologous Events
Cross References
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