Reactome | G6PC3 hydrolyzes G6P to form Glc and Pi (ubiquitous)

G6PC3 hydrolyzes G6P to form Glc and Pi (ubiquitous)

Stable Identifier

R-HSA-3262512

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen, endoplasmic reticulum membrane

Synonyms

alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC3 - ubiquitous)

ReviewStatus

5/5

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G6PC3 hydrolyzes G6P to form Glc and Pi (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 Boztug, K, Appaswamy, G, Ashikov, A, Schäffer, AA, Salzer, U, Diestelhorst, J, Germeshausen, M, Brandes, G, Lee-Gossler, J, Noyan, F, Gatzke, AK, Minkov, M, Greil, J, Kratz, C, Petropoulou, T, Pellier, I, Bellanné-Chantelot, C, Rezaei, N, Mönkemöller, K, Irani-Hakimeh, N, Bakker, H, Gerardy-Schahn, R, Zeidler, C, Grimbacher, B, Welte, K, Klein, C	N. Engl. J. Med.	2009
12965222	Identification and characterisation of a new human glucose-6-phosphatase isoform Guionie, O, Clottes, E, Stafford, K, Burchell, A	FEBS Lett	2003
14718531	Histidine 167 is the phosphate acceptor in glucose-6-phosphatase-beta forming a phosphohistidine enzyme intermediate during catalysis Ghosh, A, Shieh, JJ, Pan, CJ, Chou, JY	J. Biol. Chem.	2004