Reviewed, UniProtKB/Swiss-Prot O19133 (G6PC_CANFA)

Names and origin

Protein names

Recommended name:
    Glucose-6-phosphatase
      Short name=G-6-Pase
      Short name=G6Pase
    EC=3.1.3.9

Gene names

Name:	G6PC
Synonyms:	G6PT

Organism

Canis familiaris (Dog)

Taxonomic identifier

9615 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Laurasiatheria › Carnivora › Caniformia › Canidae › Canis

Protein attributes

Sequence length	357 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence 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 process	Gluconeogenesis
Cellular component	Endoplasmic reticulum Membrane
Disease	Disease mutation
Domain	Transmembrane
Molecular function	Hydrolase
PTM	Glycoprotein
Gene Ontology (GO)
Biological process	gluconeogenesis Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	endoplasmic reticulum membrane Inferred from electronic annotation. Source: UniProtKB-SubCell integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW
Molecular function	glucose-6-phosphatase activity Inferred from electronic annotation. Source: EC
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 357	357	Glucose-6-phosphatase		PRO_0000087410
Regions
	Topological domain	1 – 28	28	Lumenal Potential
	Transmembrane	29 – 49	21	Potential
	Topological domain	50 – 60	11	Cytoplasmic Potential
	Transmembrane	61 – 81	21	Potential
	Topological domain	82 – 117	36	Lumenal Potential
	Transmembrane	118 – 138	21	Potential
	Topological domain	139 – 147	9	Cytoplasmic Potential
	Transmembrane	148 – 168	21	Potential
	Topological domain	169 – 170	2	Lumenal Potential
	Transmembrane	171 – 191	21	Potential
	Topological domain	192 – 209	18	Cytoplasmic Potential
	Transmembrane	210 – 230	21	Potential
	Topological domain	231 – 254	24	Lumenal Potential
	Transmembrane	255 – 275	21	Potential
	Topological domain	276 – 291	16	Cytoplasmic Potential
	Transmembrane	292 – 312	21	Potential
	Topological domain	313 – 320	8	Lumenal Potential
	Transmembrane	321 – 341	21	Potential
	Topological domain	342 – 357	16	Cytoplasmic Potential
	Motif	354 – 357	4	Prevents secretion from ER Potential
Sites
	Active site	119	1	Proton donor Potential
	Active site	176	1	Nucleophile By similarity
	Binding site	83	1	Substrate Potential
	Binding site	170	1	Substrate Potential
Amino acid modifications
	Glycosylation	96	1	N-linked (GlcNAc...) By similarity
Natural variations
	Natural variant	121	1	M → I in GSD-Ia.

Sequences

	Sequence		Length	Mass (Da)
	O19133-1 [UniParc]. Last modified January 1, 1998. Version 1. Checksum: E60BFBD80485BEC1	FASTA	357	40,969
	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

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: 9259982] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GSD-IA ILE-121. Strain: Maltese.

Cross-references

Sequence databases
EMBLGenBankDDBJ	U91844 mRNA. Translation: AAB65147.1.
RefSeq	NP_001002993.1.
UniGene	Cfa.204
3D structure databases
ModBase	Search...
Genome annotation databases
Ensembl	ENSCAFG00000014653. Canis familiaris. [Contig view]
GeneID	403492.
KEGG	cfa:403492.
Phylogenomic databases
HOVERGEN	O19133.
Enzyme and pathway databases
BRENDA	3.1.3.9. 463.
Family and domain databases
InterPro	IPR016275. Glucose-6-phosphatase. IPR000326. P_Acid_Pase_2/haloperoxidase. [Graphical view]
Pfam	PF01569. PAP2. 1 hit. [Graphical view]
PIRSF	PIRSF000905. Glucose-6-phosphatase. 1 hit.
SMART	SM00014. acidPPc. 1 hit. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

G6PC_CANFA

Accession

Primary (citable) accession number: O19133

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 15, 1998
Last sequence update:	January 1, 1998
Last modified:	January 20, 2009
This is version 55 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)