Reaction: Defective G6PC3 does not hydrolyze glucose 6-phosphate
- in pathway: Severe congenital neutropenia type 4 (G6PC3)
Glucose-6-phosphatase 3 (G6PC3) associated with the endoplasmic reticulum membrane normally catalyzes the hydrolysis of glucose-6-phosphate to glucose and orthophosphate. In the body, this enzyme is ubiquitously expressed; mutations that inactivate it are associated with severe congenital neutropenia (but not with fasting hypoglycemia or lactic acidemia) (Boztug et al. 2009, 2012).
Reaction - small molecule participants:
H2O [endoplasmic reticulum lumen]
G6P [endoplasmic reticulum lumen]
Reactome.org reaction link: R-HSA-3282876
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Reaction input - small molecules:
water
alpha-D-glucose 6-phosphate(2-)
Reaction output - small molecules:
Reactome.org link: R-HSA-3282876