G6PC3 hydrolyzes G6P to form Glc and Pi (ubiquitous)

Stable Identifier
Reaction [transition]
Homo sapiens
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC3 - ubiquitous)
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Glucose-6-phosphatase 3 (G6PC3) associated with the endoplasmic reticulum membrane catalyzes the hydrolysis of glucose-6-phosphate (G6) to glucose (Glc) and orthophosphate (Pi). This reaction is essentially irreversible (Guionie et al. 2003; Ghosh et al. 2004). 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).
Literature References
PubMed ID Title Journal Year
19118303 A syndrome with congenital neutropenia and mutations in G6PC3

Klein, C, Kratz, C, Salzer, U, Brandes, G, Mönkemöller, K, Germeshausen, M, Lee-Gossler, J, Bellanné-Chantelot, C, Appaswamy, G, Minkov, M, Greil, J, Zeidler, C, Irani-Hakimeh, N, Boztug, K, Welte, K, Rezaei, N, Gatzke, AK, Petropoulou, T, Gerardy-Schahn, R, Schäffer, AA, Grimbacher, B, Noyan, F, Pellier, I, Diestelhorst, J, Bakker, H, Ashikov, A

N. Engl. J. Med. 2009
12965222 Identification and characterisation of a new human glucose-6-phosphatase isoform

Guionie, O, Clottes, E, Burchell, A, Stafford, K

FEBS Lett 2003
14718531 Histidine 167 is the phosphate acceptor in glucose-6-phosphatase-beta forming a phosphohistidine enzyme intermediate during catalysis

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

J. Biol. Chem. 2004
Catalyst Activity

glucose-6-phosphatase activity of G6PC3 [endoplasmic reticulum membrane]

Orthologous Events
Cross References
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