Reactome | G6PC hydrolyzes G6P to Glc and Pi (liver)

G6PC hydrolyzes G6P to Glc and Pi (liver)

Stable Identifier

R-HSA-71825

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen, endoplasmic reticulum membrane

Synonyms

alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC - liver)

ReviewStatus

5/5

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G6PC hydrolyzes G6P to Glc and Pi (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 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
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
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