G6PC hydrolyzes G6P to Glc and Pi (liver)
G6P + H2O → Pi + Glc
- Reactome
- R-HSA-71825
- Compartment
- endoplasmic reticulum lumen · endoplasmic reticulum membrane
- Rhea
- RHEA:44905
Inputs
- G6PCHEBI:58225, C6H11O9P, endoplasmic reticulum lumen
- H2OCHEBI:15377, H2O, endoplasmic reticulum lumen
Outputs
- PiCHEBI:43474, HO4P, endoplasmic reticulum lumen
- GlcCHEBI:4167, C6H12O6, endoplasmic reticulum lumen
Catalysed by
- glucose-6-phosphatase activity of G6PC [endoplasmic reticulum membrane]EC 3.1.3.9; UniProt P35575
Literature
- Genetic basis of glycogen storage disease type 1a: Prevalent mutations at the glucose-6-phosphatase locus Am J Hum Genet, 1995
- The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis J. Biol. Chem., 2002
- Mutations in the glucose-6-phosphatase gene that cause glycogen storage disease type 1a Science, 1993
- Mutations in the glucose-6-phosphatase-alpha (G6PC) gene that cause type Ia glycogen storage disease Hum. Mutat., 2008