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O19133

- G6PC_CANFA

UniProt

O19133 - G6PC_CANFA

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Protein
Glucose-6-phosphatase
Gene
G6PC, G6PT
Organism
Canis familiaris (Dog) (Canis lupus familiaris)
Status
Reviewed - Annotation score: 4 out of 5 - Experimental evidence at protein leveli

Functioni

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 activityi

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

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei83 – 831Substrate Reviewed prediction
Active sitei119 – 1191Proton donor Reviewed prediction
Binding sitei170 – 1701Substrate Reviewed prediction
Active sitei176 – 1761Nucleophile By similarity

GO - Molecular functioni

  1. glucose-6-phosphatase activity Source: UniProtKB-EC
Complete GO annotation...

GO - Biological processi

  1. gluconeogenesis Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Biological processi

Gluconeogenesis

Enzyme and pathway databases

UniPathwayiUPA00138.

Names & Taxonomyi

Protein namesi
Recommended name:
Glucose-6-phosphatase (EC:3.1.3.9)
Short name:
G-6-Pase
Short name:
G6Pase
Gene namesi
Name:G6PC
Synonyms:G6PT
OrganismiCanis familiaris (Dog) (Canis lupus familiaris)
Taxonomic identifieri9615 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCarnivoraCaniformiaCanidaeCanis
ProteomesiUP000002254: Unplaced

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 2828Lumenal Reviewed prediction
Add
BLAST
Transmembranei29 – 4921Helical; Reviewed prediction
Add
BLAST
Topological domaini50 – 6011Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei61 – 8121Helical; Reviewed prediction
Add
BLAST
Topological domaini82 – 11736Lumenal Reviewed prediction
Add
BLAST
Transmembranei118 – 13821Helical; Reviewed prediction
Add
BLAST
Topological domaini139 – 1479Cytoplasmic Reviewed prediction
Transmembranei148 – 16821Helical; Reviewed prediction
Add
BLAST
Topological domaini169 – 1702Lumenal Reviewed prediction
Transmembranei171 – 19121Helical; Reviewed prediction
Add
BLAST
Topological domaini192 – 20918Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei210 – 23021Helical; Reviewed prediction
Add
BLAST
Topological domaini231 – 25424Lumenal Reviewed prediction
Add
BLAST
Transmembranei255 – 27521Helical; Reviewed prediction
Add
BLAST
Topological domaini276 – 29116Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei292 – 31221Helical; Reviewed prediction
Add
BLAST
Topological domaini313 – 3208Lumenal Reviewed prediction
Transmembranei321 – 34121Helical; Reviewed prediction
Add
BLAST
Topological domaini342 – 35716Cytoplasmic Reviewed prediction
Add
BLAST

GO - Cellular componenti

  1. endoplasmic reticulum membrane Source: UniProtKB-SubCell
  2. integral component of membrane Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

Pathology & Biotechi

Involvement in diseasei

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.

Keywords - Diseasei

Disease mutation

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 357357Glucose-6-phosphatase
PRO_0000087410Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi96 – 961N-linked (GlcNAc...) By similarity

Keywords - PTMi

Glycoprotein

Interactioni

Protein-protein interaction databases

STRINGi9615.ENSCAFP00000021606.

Structurei

3D structure databases

ProteinModelPortaliO19133.

Family & Domainsi

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi354 – 3574Prevents secretion from ER Reviewed prediction

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG82628.
HOGENOMiHOG000264239.
HOVERGENiHBG003560.
InParanoidiO19133.
KOiK01084.

Family and domain databases

Gene3Di1.20.144.10. 1 hit.
InterProiIPR016275. Glucose-6-phosphatase.
IPR000326. P_Acid_Pase_2/haloperoxidase.
[Graphical view]
PfamiPF01569. PAP2. 1 hit.
[Graphical view]
PIRSFiPIRSF000905. Glucose-6-phosphatase. 1 hit.
SMARTiSM00014. acidPPc. 1 hit.
[Graphical view]
SUPFAMiSSF48317. SSF48317. 1 hit.

Sequencei

Sequence statusi: Complete.

O19133-1 [UniParc]FASTAAdd to Basket

« Hide

MEKGMDVLHD FGIQSTHYLQ VNYQDSQDWF ILVSVIADLR NAFYVLFPIW    50
FHLREAVGIK LLWVAVIGDW LNLVFKWILF GQRPYWWVMD TDYYSNTSVP 100
LIKQFPVTCE TGPGSPSGHA MGTAGVYYVM VTSTLSIFRG RKRPTYRFRC 150
LNILLWLGFW AVQLNVCLSR IYLAAHFPHQ VVAGVLSGIA VAETFRHIQS 200
IYNASLKKYF LITFFLFSFA IGFYLLLKGL GVDLLWTLEK ARRWCERPEW 250
VHIDTTPFAS LLKNVGTLFG LGVTLNSSMY RESCKGKLSK WFPFRLSCIV 300
VSLILLHLFD SLKPPSQTEL IFYTLSFCKS AAVPLASVSL IPYCLARVFD 350
QPDKKSL 357
Length:357
Mass (Da):40,969
Last modified:January 1, 1998 - v1
Checksum:iE60BFBD80485BEC1
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti121 – 1211M → I in GSD-Ia. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U91844 mRNA. Translation: AAB65147.1.
RefSeqiNP_001002993.1. NM_001002993.1.
UniGeneiCfa.204.

Genome annotation databases

GeneIDi403492.
KEGGicfa:403492.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U91844 mRNA. Translation: AAB65147.1 .
RefSeqi NP_001002993.1. NM_001002993.1.
UniGenei Cfa.204.

3D structure databases

ProteinModelPortali O19133.
ModBasei Search...

Protein-protein interaction databases

STRINGi 9615.ENSCAFP00000021606.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

GeneIDi 403492.
KEGGi cfa:403492.

Organism-specific databases

CTDi 2538.

Phylogenomic databases

eggNOGi NOG82628.
HOGENOMi HOG000264239.
HOVERGENi HBG003560.
InParanoidi O19133.
KOi K01084.

Enzyme and pathway databases

UniPathwayi UPA00138 .

Miscellaneous databases

NextBioi 20817007.

Family and domain databases

Gene3Di 1.20.144.10. 1 hit.
InterProi IPR016275. Glucose-6-phosphatase.
IPR000326. P_Acid_Pase_2/haloperoxidase.
[Graphical view ]
Pfami PF01569. PAP2. 1 hit.
[Graphical view ]
PIRSFi PIRSF000905. Glucose-6-phosphatase. 1 hit.
SMARTi SM00014. acidPPc. 1 hit.
[Graphical view ]
SUPFAMi SSF48317. SSF48317. 1 hit.
ProtoNeti Search...

Publicationsi

  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.

Entry informationi

Entry nameiG6PC_CANFA
AccessioniPrimary (citable) accession number: O19133
Entry historyi
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 statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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