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O19133

- G6PC_CANFA

UniProt

O19133 - G6PC_CANFA

Protein

Glucose-6-phosphatase

Gene

G6PC

Organism
Canis familiaris (Dog) (Canis lupus familiaris)
Status
Reviewed - Annotation score: 4 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 87 (01 Oct 2014)
      Sequence version 1 (01 Jan 1998)
      Previous versions | rss
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    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.By similarity

    Catalytic activityi

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

    Pathwayi

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei83 – 831SubstrateSequence Analysis
    Active sitei119 – 1191Proton donorSequence Analysis
    Binding sitei170 – 1701SubstrateSequence Analysis
    Active sitei176 – 1761NucleophileBy similarity

    GO - Molecular functioni

    1. glucose-6-phosphatase activity Source: UniProtKB-EC

    GO - Biological processi

    1. gluconeogenesis Source: UniProtKB-UniPathway

    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

    GO - Cellular componenti

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

    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-phosphatasePRO_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.
    ModBaseiSearch...
    MobiDBiSearch...

    Topological domain

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Topological domaini1 – 2828LumenalSequence AnalysisAdd
    BLAST
    Topological domaini50 – 6011CytoplasmicSequence AnalysisAdd
    BLAST
    Topological domaini82 – 11736LumenalSequence AnalysisAdd
    BLAST
    Topological domaini139 – 1479CytoplasmicSequence Analysis
    Topological domaini169 – 1702LumenalSequence Analysis
    Topological domaini192 – 20918CytoplasmicSequence AnalysisAdd
    BLAST
    Topological domaini231 – 25424LumenalSequence AnalysisAdd
    BLAST
    Topological domaini276 – 29116CytoplasmicSequence AnalysisAdd
    BLAST
    Topological domaini313 – 3208LumenalSequence Analysis
    Topological domaini342 – 35716CytoplasmicSequence AnalysisAdd
    BLAST

    Transmembrane

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transmembranei29 – 4921HelicalSequence AnalysisAdd
    BLAST
    Transmembranei61 – 8121HelicalSequence AnalysisAdd
    BLAST
    Transmembranei118 – 13821HelicalSequence AnalysisAdd
    BLAST
    Transmembranei148 – 16821HelicalSequence AnalysisAdd
    BLAST
    Transmembranei171 – 19121HelicalSequence AnalysisAdd
    BLAST
    Transmembranei210 – 23021HelicalSequence AnalysisAdd
    BLAST
    Transmembranei255 – 27521HelicalSequence AnalysisAdd
    BLAST
    Transmembranei292 – 31221HelicalSequence AnalysisAdd
    BLAST
    Transmembranei321 – 34121HelicalSequence AnalysisAdd
    BLAST

    Family & Domainsi

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi354 – 3574Prevents secretion from ERSequence Analysis

    Sequence similaritiesi

    Belongs to the glucose-6-phosphatase family.Curated

    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

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    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...
    MobiDBi 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: October 1, 2014
    This is version 87 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