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MetaCyc Enzyme: glucose-6-phosphatase

Gene: G6PC Accession Number: HS05538 (MetaCyc)

Synonyms: G6PT, G-6-Pase, G6Pase, G6Pase-alpha, glucose-6-phosphatase alpha

Species: Homo sapiens

Summary:
Glucose-6-phosphatase (G6Pase) is an integral membrane protein of the endoplasmic reticulum that catalyzes the hydrolysis of D-glucose 6-phosphate to D-glucose and orthophosphate [Ghosh02]. There are three known isoforms of the enzyme glucose-6-phosphatase, glucose-6-phosphatase 2 and glucose-6-phosphatase 3. It is a key enzyme in glucose homeostasis, functioning in both gluconeogenesis and glycogenolysis.

Studies of G6Pase in the primary gluconeogenic tissue of liver and kidney revealed that G6Pase forms a complex with the glucose-6-phosphate transporter and their activities are tightly coupled [Lei96]. Defects in the glucose-6-phosphate transporter causes glycogen storage disease type 1b [Hiraiwa99]. Defects in the enzyme cause glycogen storage disease type Ia (von Gierke disease) [Lei93][Shieh02].

Locations: endoplasmic reticulum lumen, membrane, endoplasmic reticulum

Map Position: [43,214,753 -> 43,227,336]

Molecular Weight of Polypeptide: 40.514 kD (from nucleotide sequence)

Unification Links: ArrayExpress:P35575 , Ensembl:ENSG00000131482 , Entrez-gene:2538 , Entrez-Nucleotide:AF097831 , Entrez-Nucleotide:U01120 , Entrez:AAA16222 , Entrez:AAD19898 , Entrez:AAH20700 , GeneCards:G6PC , OMIM:232200 , PhosphoSite:P35575 , Pride:P35575 , Protein Model Portal:P35575 , RefSeq:NM_000151 , RefSeq:NP_000142 , String:9606.ENSP00000253801 , UCSC Human Genome:NM_000151 , UniGene:242 , UniProt:P35575

Relationship Links: InterPro:IN-FAMILY:IPR000326 , InterPro:IN-FAMILY:IPR016275 , Pfam:IN-FAMILY:PF01569 , Smart:IN-FAMILY:SM00014

Gene-Reaction Schematic: ?

Instance reactions of [D-glucopyranose 6-phosphate + H2O → D-glucopyranose + phosphate] (3.1.3.9/3.1.3.58):
i1: α-D-glucose 6-phosphate + H2O → α-D-glucose + phosphate (3.1.3.9/3.1.3.58)

i2: β-D-glucose 6-phosphate + H2O → β-D-glucose + phosphate (3.1.3.9/3.1.3.58)

GO Terms:

Biological Process: GO:0005977 - glycogen metabolic process
GO:0005978 - glycogen biosynthetic process
Molecular Function: GO:0004346 - glucose-6-phosphatase activity
GO:0016787 - hydrolase activity
Cellular Component: GO:0005783 - endoplasmic reticulum
GO:0005788 - endoplasmic reticulum lumen
GO:0016021 - integral component of membrane


Enzymatic reaction of: glucose-6-phosphatase

EC Number: 3.1.3.9/3.1.3.58

D-glucopyranose 6-phosphate + H2O <=> D-glucopyranose + phosphate

The reaction direction shown, that is, A + B ↔ C + D versus C + D ↔ A + B, is in accordance with the Enzyme Commission system.

The reaction is physiologically favored in the direction shown.

In Pathways: gluconeogenesis III

Credits:
Imported from HumanCyc 18-Mar-2013 by Caspi R , SRI International


Enzymatic reaction of: gluocose-6-phosphatase (glucose-6-phosphatase)

EC Number: 3.1.3.9/3.1.3.58

β-D-glucose 6-phosphate + H2O <=> β-D-glucose + phosphate

The reaction direction shown, that is, A + B ↔ C + D versus C + D ↔ A + B, is in accordance with the Enzyme Commission system.

The reaction is physiologically favored in the direction shown.


References

Ghosh02: Ghosh A, Shieh JJ, Pan CJ, Sun MS, Chou JY (2002). "The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis." J Biol Chem 277(36);32837-42. PMID: 12093795

Hiraiwa99: Hiraiwa H, Pan CJ, Lin B, Moses SW, Chou JY (1999). "Inactivation of the glucose 6-phosphate transporter causes glycogen storage disease type 1b." J Biol Chem 274(9);5532-6. PMID: 10026167

Lei93: Lei KJ, Shelly LL, Pan CJ, Sidbury JB, Chou JY (1993). "Mutations in the glucose-6-phosphatase gene that cause glycogen storage disease type 1a." Science 262(5133);580-3. PMID: 8211187

Lei96: Lei KJ, Chen H, Pan CJ, Ward JM, Mosinger B, Lee EJ, Westphal H, Mansfield BC, Chou JY (1996). "Glucose-6-phosphatase dependent substrate transport in the glycogen storage disease type-1a mouse." Nat Genet 13(2);203-9. PMID: 8640227

Shieh02: Shieh JJ, Terzioglu M, Hiraiwa H, Marsh J, Pan CJ, Chen LY, Chou JY (2002). "The molecular basis of glycogen storage disease type 1a: structure and function analysis of mutations in glucose-6-phosphatase." J Biol Chem 277(7);5047-53. PMID: 11739393


Report Errors or Provide Feedback
Please cite the following article in publications resulting from the use of MetaCyc: Caspi et al, Nucleic Acids Research 42:D459-D471 2014
Page generated by SRI International Pathway Tools version 18.5 on Mon Jan 26, 2015, BIOCYC14B.