Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

O19133 (G6PC_CANFA) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 86. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Glucose-6-phosphatase

Short name=G-6-Pase
Short name=G6Pase
EC=3.1.3.9
Gene names
Name:G6PC
Synonyms:G6PT
OrganismCanis familiaris (Dog) (Canis lupus familiaris) [Reference proteome]
Taxonomic identifier9615 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCarnivoraCaniformiaCanidaeCanis

Protein attributes

Sequence length357 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production through glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels By similarity.

Catalytic activity

D-glucose 6-phosphate + H2O = D-glucose + phosphate.

Pathway

Carbohydrate biosynthesis; gluconeogenesis.

Subcellular location

Endoplasmic reticulum membrane; Multi-pass membrane protein.

Involvement in disease

Defects in G6PC are the cause of glycogen storage disease Ia (GSD-Ia); also known as von Gierke disease. GSD-Ia is characterized by hypoglycemia and hepatomegaly.

Sequence similarities

Belongs to the glucose-6-phosphatase family.

Ontologies

Keywords
   Biological processGluconeogenesis
   Cellular componentEndoplasmic reticulum
Membrane
   DiseaseDisease mutation
   DomainTransmembrane
Transmembrane helix
   Molecular functionHydrolase
   PTMGlycoprotein
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processgluconeogenesis

Inferred from electronic annotation. Source: UniProtKB-UniPathway

   Cellular_componentendoplasmic reticulum membrane

Inferred from electronic annotation. Source: UniProtKB-SubCell

integral component of membrane

Inferred from electronic annotation. Source: UniProtKB-KW

   Molecular_functionglucose-6-phosphatase activity

Inferred from electronic annotation. Source: UniProtKB-EC

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 357357Glucose-6-phosphatase
PRO_0000087410

Regions

Topological domain1 – 2828Lumenal Potential
Transmembrane29 – 4921Helical; Potential
Topological domain50 – 6011Cytoplasmic Potential
Transmembrane61 – 8121Helical; Potential
Topological domain82 – 11736Lumenal Potential
Transmembrane118 – 13821Helical; Potential
Topological domain139 – 1479Cytoplasmic Potential
Transmembrane148 – 16821Helical; Potential
Topological domain169 – 1702Lumenal Potential
Transmembrane171 – 19121Helical; Potential
Topological domain192 – 20918Cytoplasmic Potential
Transmembrane210 – 23021Helical; Potential
Topological domain231 – 25424Lumenal Potential
Transmembrane255 – 27521Helical; Potential
Topological domain276 – 29116Cytoplasmic Potential
Transmembrane292 – 31221Helical; Potential
Topological domain313 – 3208Lumenal Potential
Transmembrane321 – 34121Helical; Potential
Topological domain342 – 35716Cytoplasmic Potential
Motif354 – 3574Prevents secretion from ER Potential

Sites

Active site1191Proton donor Potential
Active site1761Nucleophile By similarity
Binding site831Substrate Potential
Binding site1701Substrate Potential

Amino acid modifications

Glycosylation961N-linked (GlcNAc...) By similarity

Natural variations

Natural variant1211M → I in GSD-Ia. Ref.1

Sequences

Sequence LengthMass (Da)Tools
O19133 [UniParc].

Last modified January 1, 1998. Version 1.
Checksum: E60BFBD80485BEC1

FASTA35740,969
        10         20         30         40         50         60 
MEKGMDVLHD FGIQSTHYLQ VNYQDSQDWF ILVSVIADLR NAFYVLFPIW FHLREAVGIK 

        70         80         90        100        110        120 
LLWVAVIGDW LNLVFKWILF GQRPYWWVMD TDYYSNTSVP LIKQFPVTCE TGPGSPSGHA 

       130        140        150        160        170        180 
MGTAGVYYVM VTSTLSIFRG RKRPTYRFRC LNILLWLGFW AVQLNVCLSR IYLAAHFPHQ 

       190        200        210        220        230        240 
VVAGVLSGIA VAETFRHIQS IYNASLKKYF LITFFLFSFA IGFYLLLKGL GVDLLWTLEK 

       250        260        270        280        290        300 
ARRWCERPEW VHIDTTPFAS LLKNVGTLFG LGVTLNSSMY RESCKGKLSK WFPFRLSCIV 

       310        320        330        340        350 
VSLILLHLFD SLKPPSQTEL IFYTLSFCKS AAVPLASVSL IPYCLARVFD QPDKKSL 

« Hide

References

[1]"Isolation and nucleotide sequence of canine glucose-6-phosphatase mRNA: identification of mutation in puppies with glycogen storage disease type Ia."
Kishnani P.S., Bao Y., Wu J.Y., Brix A.E., Lin J.L., Chen Y.T.
Biochem. Mol. Med. 61:168-177(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GSD-IA ILE-121.
Strain: Maltese.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U91844 mRNA. Translation: AAB65147.1.
RefSeqNP_001002993.1. NM_001002993.1.
UniGeneCfa.204.

3D structure databases

ProteinModelPortalO19133.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

STRING9615.ENSCAFP00000021606.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

GeneID403492.
KEGGcfa:403492.

Organism-specific databases

CTD2538.

Phylogenomic databases

eggNOGNOG82628.
HOGENOMHOG000264239.
HOVERGENHBG003560.
InParanoidO19133.
KOK01084.

Enzyme and pathway databases

UniPathwayUPA00138.

Family and domain databases

Gene3D1.20.144.10. 1 hit.
InterProIPR016275. Glucose-6-phosphatase.
IPR000326. P_Acid_Pase_2/haloperoxidase.
[Graphical view]
PfamPF01569. PAP2. 1 hit.
[Graphical view]
PIRSFPIRSF000905. Glucose-6-phosphatase. 1 hit.
SMARTSM00014. acidPPc. 1 hit.
[Graphical view]
SUPFAMSSF48317. SSF48317. 1 hit.
ProtoNetSearch...

Other

NextBio20817007.

Entry information

Entry nameG6PC_CANFA
AccessionPrimary (citable) accession number: O19133
Entry history
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 1, 1998
Last modified: April 16, 2014
This is version 86 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

PATHWAY comments

Index of metabolic and biosynthesis pathways