UniProtKB - P35575 (G6PC_HUMAN)

BLAST

>sp|P35575|G6PC_HUMAN Glucose-6-phosphatase OS=Homo sapiens OX=9606 GN=G6PC PE=1 SV=2
MEEGMNVLHDFGIQSTHYLQVNYQDSQDWFILVSVIADLRNAFYVLFPIWFHLQEAVGIK
LLWVAVIGDWLNLVFKWILFGQRPYWWVLDTDYYSNTSVPLIKQFPVTCETGPGSPSGHA
MGTAGVYYVMVTSTLSIFQGKIKPTYRFRCLNVILWLGFWAVQLNVCLSRIYLAAHFPHQ
VVAGVLSGIAVAETFSHIHSIYNASLKKYFLITFFLFSFAIGFYLLLKGLGVDLLWTLEK
AQRWCEQPEWVHIDTTPFASLLKNLGTLFGLGLALNSSMYRESCKGKLSKWLPFRLSSIV
ASLVLLHVFDSLKPPSQVELVFYVLSFCKSAVVPLASVSVIPYCLAQVLGQPHKKSL

Align

Format
Add to basket Added to basket
History
Entry version 175 (07 Nov 2018)
Sequence version 2 (23 Sep 2008)
Previous versions | rss
Other tutorials and videos
Help video
Feedback

Protein

Glucose-6-phosphatase

Gene

G6PC

Organism

Homo sapiens (Human)

Status

Reviewed-Annotation score: -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.

Catalytic activityⁱ

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

Pathwayⁱ: gluconeogenesis

This protein is involved in the pathway gluconeogenesis, which is part of Carbohydrate biosynthesis.
View all proteins of this organism that are known to be involved in the pathway gluconeogenesis and in Carbohydrate biosynthesis.

Sites

Feature key	Position(s)	DescriptionActions	Length
Binding siteⁱ	83	SubstrateSequence analysis	1
Active siteⁱ	119	Proton donorSequence analysis	1
Binding siteⁱ	170	SubstrateSequence analysis	1
Active siteⁱ	176	Nucleophile1 Publication	1

GO - Molecular functionⁱ

glucose-6-phosphatase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 10318794
- 8211187
phosphate ion binding
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 12093795
phosphotransferase activity, alcohol group as acceptor Source: Ensembl

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

cholesterol homeostasis Source: Ensembl
gluconeogenesis
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 8211187
glucose 6-phosphate metabolic process
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
glucose-6-phosphate transport Source: Ensembl
glucose homeostasis
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 8211187
glycogen catabolic process Source: Ensembl
glycogen metabolic process
Source: ProtInc
Traceable author statementⁱ
- 8211187
multicellular organism growth Source: Ensembl
phosphorylated carbohydrate dephosphorylation Source: Ensembl
regulation of gene expression Source: Ensembl
response to food Source: Ensembl
steroid metabolic process Source: Ensembl
triglyceride metabolic process Source: Ensembl
urate metabolic process Source: Ensembl

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Hydrolase
Biological process	Gluconeogenesis

Enzyme and pathway databases

BioCycⁱ	MetaCyc:HS05538-MONOMER
BRENDAⁱ	3.1.3.9 2681
Reactomeⁱ	R-HSA-3274531 Glycogen storage disease type Ia (G6PC) R-HSA-70263 Gluconeogenesis
SABIO-RKⁱ	P35575
SIGNORⁱ	P35575
UniPathwayⁱ	UPA00138

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Glucose-6-phosphatase (EC:3.1.3.9) Short name: G-6-Pase Short name: G6Pase Alternative name(s): Glucose-6-phosphatase alpha Short name: G6Pase-alpha
Gene namesⁱ	Name:G6PC Synonyms:G6PT
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 17

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000131482.9
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:4056 G6PC
MIMⁱ	613742 gene
neXtProtⁱ	NX_P35575

Topology

Feature key	Position(s)	DescriptionActions	Length
Topological domainⁱ	1 – 28	LumenalSequence analysisAdd BLAST	28
Transmembraneⁱ	29 – 49	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	50 – 60	CytoplasmicSequence analysisAdd BLAST	11
Transmembraneⁱ	61 – 81	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	82 – 117	LumenalSequence analysisAdd BLAST	36
Transmembraneⁱ	118 – 138	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	139 – 147	CytoplasmicSequence analysis	9
Transmembraneⁱ	148 – 168	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	169 – 179	LumenalSequence analysisAdd BLAST	11
Transmembraneⁱ	180 – 202	HelicalSequence analysisAdd BLAST	23
Topological domainⁱ	203 – 209	CytoplasmicSequence analysis	7
Transmembraneⁱ	210 – 230	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	231 – 254	LumenalSequence analysisAdd BLAST	24
Transmembraneⁱ	255 – 275	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	276 – 291	CytoplasmicSequence analysisAdd BLAST	16
Transmembraneⁱ	292 – 312	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	313 – 320	LumenalSequence analysis	8
Transmembraneⁱ	321 – 341	HelicalSequence analysisAdd BLAST	21
Topological domainⁱ	342 – 357	CytoplasmicSequence analysisAdd BLAST	16

Keywords - Cellular componentⁱ

Endoplasmic reticulum, Membrane

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Glycogen storage disease 1A (GSD1A)24 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionA metabolic disorder characterized by impairment of terminal steps of glycogenolysis and gluconeogenesis. Patients manifest a wide range of clinical symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia.

Mutagenesis

Feature key	Position(s)	DescriptionActions	Length
Mutagenesisⁱ	9	H → A: Partial loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	52	H → A: Partial loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	119	H → A: Loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	176	H → A: Loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	179	H → A: Loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	197	H → T: Partial loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	252	H → A: Partial loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	307	H → A: Partial loss of catalytic activity. 1 Publication	1
Mutagenesisⁱ	353	H → A: Partial loss of catalytic activity. 1 Publication	1

Keywords - Diseaseⁱ

Disease mutation, Glycogen storage disease

Organism-specific databases

DisGeNET More...DisGeNETⁱ	2538
GeneReviewsⁱ	G6PC
MalaCards human disease database More...MalaCardsⁱ	G6PC
MIMⁱ	232200 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000131482
Orphanetⁱ	79258 Glycogen storage disease due to glucose-6-phosphatase deficiency type Ia
PharmGKBⁱ	PA28468

Chemistry databases

ChEMBLⁱ	CHEMBL2282

ChEMBLⁱ

CHEMBL2282

Polymorphism and mutation databases

BioMutaⁱ	G6PC
DMDMⁱ	206729864

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000087413	1 – 357	Glucose-6-phosphataseAdd BLAST		357

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Glycosylationⁱ	96	N-linked (GlcNAc...) asparagine2 Publications		1

Keywords - PTMⁱ

Glycoprotein

Proteomic databases

PaxDbⁱ	P35575
PeptideAtlas More...PeptideAtlasⁱ	P35575
PRIDEⁱ	P35575
ProteomicsDBⁱ	55092

PTM databases

DEPODⁱ	P35575
GlyConnectⁱ	1267
iPTMnetⁱ	P35575
PhosphoSitePlusⁱ	P35575

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000131482 Expressed in 58 organ(s), highest expression level in liver
CleanExⁱ	HS_G6PC
ExpressionAtlasⁱ	P35575 baseline and differential
Genevisibleⁱ	P35575 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA052324

HPAⁱ

HPA052324

Interactionⁱ

Protein-protein interaction databases

BioGridⁱ	108813, 3 interactors
IntActⁱ	P35575, 3 interactors
STRINGⁱ	9606.ENSP00000253801

Chemistry databases

BindingDBⁱ

P35575

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	P35575
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the glucose-6-phosphatase family.Curated

Keywords - Domainⁱ

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGⁱ	ENOG410IDXG Eukaryota ENOG4110AJ7 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00510000046465
HOGENOMⁱ	HOG000264239
HOVERGENⁱ	HBG003560
InParanoidⁱ	P35575
KEGG Orthology (KO) More...KOⁱ	K01084
OMAⁱ	RYLQVNY
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0AXF
PhylomeDBⁱ	P35575
TreeFamⁱ	TF324388

Family and domain databases

InterProⁱ	View protein in InterPro IPR016275 Glucose-6-phosphatase IPR036938 P_Acid_Pase_2/haloperoxi_sf IPR000326 P_Acid_Pase_2/haloperoxidase
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF01569 PAP2, 1 hit
PIRSFⁱ	PIRSF000905 Glucose-6-phosphatase, 1 hit
SMARTⁱ	View protein in SMART SM00014 acidPPc, 1 hit
SUPFAMⁱ	SSF48317 SSF48317, 1 hit

Sequences (2+)ⁱ

Sequence statusⁱ: Complete.

This entry describes 2 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show all Align All

Isoform 1 (identifier: P35575-1) [UniParc]FASTA Add to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MEEGMNVLHD FGIQSTHYLQ VNYQDSQDWF ILVSVIADLR NAFYVLFPIW 
        60         70         80         90        100
FHLQEAVGIK LLWVAVIGDW LNLVFKWILF GQRPYWWVLD TDYYSNTSVP 
       110        120        130        140        150
LIKQFPVTCE TGPGSPSGHA MGTAGVYYVM VTSTLSIFQG KIKPTYRFRC 
       160        170        180        190        200
LNVILWLGFW AVQLNVCLSR IYLAAHFPHQ VVAGVLSGIA VAETFSHIHS 
       210        220        230        240        250
IYNASLKKYF LITFFLFSFA IGFYLLLKGL GVDLLWTLEK AQRWCEQPEW 
       260        270        280        290        300
VHIDTTPFAS LLKNLGTLFG LGLALNSSMY RESCKGKLSK WLPFRLSSIV 
       310        320        330        340        350
ASLVLLHVFD SLKPPSQVEL VFYVLSFCKS AVVPLASVSV IPYCLAQVLG 

QPHKKSL

Length:357

Mass (Da):40,484

Last modified:September 23, 2008 - v2

Checksum:ⁱ2FEA1C78928A9919

Isoform 2 (identifier: P35575-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
115-175: SPSGHAMGTA...VCLSRIYLAA → KDKADLQISV...CRNFQPHPQH
176-356: Missing.

« Hide

        10         20         30         40         50
MEEGMNVLHD FGIQSTHYLQ VNYQDSQDWF ILVSVIADLR NAFYVLFPIW 
        60         70         80         90        100
FHLQEAVGIK LLWVAVIGDW LNLVFKWILF GQRPYWWVLD TDYYSNTSVP 
       110        120        130        140        150
LIKQFPVTCE TGPGKDKADL QISVLECHFV VGILGCAAEC LSVTNLPCCS 
       160        170 
FSSSSCCWSP VRHCCCRNFQ PHPQHL

Note: No experimental confirmation available.

Show »

Length:176

Mass (Da):20,209

Checksum:ⁱ9185BD4F9911B02D

Computationally mapped potential isoform sequencesⁱ

There is 1 potential isoform mapped to this entry.BLAST Align Show all Add to basket

	Entry	Entry name		Protein names	Gene names	Length	Annotation

	K7ELS6	K7ELS6_HUMAN		Glucose-6-phosphatase Glucose-6-phosphatase	G6PC	163	Annotation score:

Sequence cautionⁱ

The sequence BAG64735 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	109	C → R in BAG64735 (PubMed:14702039).Curated		1
Sequence conflictⁱ	192	A → T in AAA16222 (PubMed:8211187).Curated		1

Natural variant

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_046249	5	M → R in GSD1A. 1 Publication	1
Natural variantⁱVAR_046250	16	T → A in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs761839506Ensembl.	1
Natural variantⁱVAR_046251	16	T → R in GSD1A; complete loss of activity and reduced enzyme stability. 1 Publication	1
Natural variantⁱVAR_009202	20	Q → R in GSD1A. 2 Publications	1
Natural variantⁱVAR_005237	38	D → V in GSD1A. 4 PublicationsCorresponds to variant dbSNP:rs104894565EnsemblClinVar.	1
Natural variantⁱVAR_009203	54	Q → P in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs1057517008Ensembl.	1
Natural variantⁱVAR_046252	63	W → R in GSD1A. 1 Publication	1
Natural variantⁱVAR_046253	65	A → P in GSD1A. 1 Publication	1
Natural variantⁱVAR_046254	68	G → R in GSD1A. 2 Publications	1
Natural variantⁱVAR_046255	76	K → N in GSD1A; loss of catalytic activity. 2 Publications	1
Natural variantⁱVAR_005238	77	W → R in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs104894566EnsemblClinVar.	1
Natural variantⁱVAR_009204	81	G → R in GSD1A. 2 PublicationsCorresponds to variant dbSNP:rs756632286Ensembl.	1
Natural variantⁱVAR_005239	83	R → C in GSD1A; complete loss of activity; prevalent mutation in Ashkenazi Jewish population. 12 PublicationsCorresponds to variant dbSNP:rs1801175EnsemblClinVar.	1
Natural variantⁱVAR_005240	83	R → H in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs1801176EnsemblClinVar.	1
Natural variantⁱVAR_005241	83	R → I in GSD1A. 1 Publication	1
Natural variantⁱVAR_009205	108	T → I in GSD1A. 2 Publications	1
Natural variantⁱVAR_005242	110	E → K in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs104894567EnsemblClinVar.	1
Natural variantⁱVAR_046256	111	T → I in GSD1A. 1 Publication	1
Natural variantⁱVAR_046257	113	P → L in GSD1A. 1 Publication	1
Natural variantⁱVAR_035922	116	P → L in a breast cancer sample; somatic mutation. 1 Publication	1
Natural variantⁱVAR_046258	119	H → L in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs1401928680Ensembl.	1
Natural variantⁱVAR_046259	122	G → D in GSD1A. 2 PublicationsCorresponds to variant dbSNP:rs759982943Ensembl.	1
Natural variantⁱVAR_005243	124	A → T in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs104894568EnsemblClinVar.	1
Natural variantⁱVAR_009206	156	W → L in GSD1A. 2 Publications	1
Natural variantⁱVAR_046260	166	V → A in GSD1A. 1 Publication	1
Natural variantⁱVAR_005244	166	V → G in GSD1A; complete loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs104894571EnsemblClinVar.	1
Natural variantⁱVAR_046261	170	R → Q in GSD1A; loss of catalytic activity. 3 PublicationsCorresponds to variant dbSNP:rs750470654Ensembl.	1
Natural variantⁱVAR_046262	177	F → C in GSD1A. 1 Publication	1
Natural variantⁱVAR_065164	178	P → A in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs763543607Ensembl.	1
Natural variantⁱVAR_046263	178	P → S in GSD1A. 1 Publication	1
Natural variantⁱVAR_046264	179	H → P in GSD1A. 1 Publication	1
Natural variantⁱVAR_005245	184	G → E in GSD1A. 2 PublicationsCorresponds to variant dbSNP:rs104894569EnsemblClinVar.	1
Natural variantⁱVAR_046265	184	G → V in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs104894569EnsemblClinVar.	1
Natural variantⁱVAR_009207	188	G → D in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs760981149Ensembl.	1
Natural variantⁱVAR_005246	188	G → R in GSD1A; complete loss of activity. 5 PublicationsCorresponds to variant dbSNP:rs80356482EnsemblClinVar.	1
Natural variantⁱVAR_046266	188	G → S in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs80356482EnsemblClinVar.	1
Natural variantⁱVAR_046268	209	Y → C in GSD1A; complete loss of activity and reduced enzyme stability. 1 Publication	1
Natural variantⁱVAR_005247	211	L → P in GSD1A. 1 Publication	1
Natural variantⁱVAR_005248	222	G → R in GSD1A. 1 Publication	1
Natural variantⁱVAR_046269	236	W → R in GSD1A. 1 Publication	1
Natural variantⁱVAR_046270	241	A → T in GSD1A. 1 Publication	1
Natural variantⁱVAR_065165	255	T → I in GSD1A. 1 Publication	1
Natural variantⁱVAR_046271	257	P → L in GSD1A. 1 Publication	1
Natural variantⁱVAR_046272	264	N → K in GSD1A. 1 Publication	1
Natural variantⁱVAR_046273	265	L → P in GSD1A. 1 Publication	1
Natural variantⁱVAR_005249	266	G → V in GSD1A. 1 Publication	1
Natural variantⁱVAR_046274	270	G → R in GSD1A. 1 Publication	1
Natural variantⁱVAR_005250	270	G → V in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs80356483EnsemblClinVar.	1
Natural variantⁱVAR_046275	270	G → W in GSD1A. 1 Publication	1
Natural variantⁱVAR_005251	295	R → C in GSD1A. 2 PublicationsCorresponds to variant dbSNP:rs104894563EnsemblClinVar.	1
Natural variantⁱVAR_046276	298	S → P in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs770003650Ensembl.	1
Natural variantⁱVAR_046277	322	F → L in GSD1A. 1 Publication	1
Natural variantⁱVAR_005252	327	Missing in GSD1A. 2 Publications	1
Natural variantⁱVAR_005253	338	V → F in GSD1A. 3 PublicationsCorresponds to variant dbSNP:rs367727229EnsemblClinVar.	1
Natural variantⁱVAR_005254	341	I → N in GSD1A. 1 PublicationCorresponds to variant dbSNP:rs387906505EnsemblClinVar.	1
Natural variantⁱVAR_046278	345	L → R in GSD1A. 1 Publication	1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Alternative sequenceⁱVSP_047558	115 – 175	SPSGH…IYLAA → KDKADLQISVLECHFVVGIL GCAAECLSVTNLPCCSFSSS SCCWSPVRHCCCRNFQPHPQ H in isoform 2. 1 PublicationAdd BLAST		61
Alternative sequenceⁱVSP_047559	176 – 356	Missing in isoform 2. 1 PublicationAdd BLAST		181

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	U01120 mRNA Translation: AAA16222.1 AK303771 mRNA Translation: BAG64735.1 Different initiation. AK313982 mRNA Translation: BAG36695.1 AC016889 Genomic DNA No translation available. BC130478 mRNA Translation: AAI30479.1 BC136369 mRNA Translation: AAI36370.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS11446.1 [P35575-1] CCDS59291.1 [P35575-2]
PIRⁱ	A48251
NCBI Reference Sequences More...RefSeqⁱ	NP_000142.2, NM_000151.3 [P35575-1] NP_001257326.1, NM_001270397.1 [P35575-2]
UniGeneⁱ	Hs.212293 Hs.742566

Genome annotation databases

Ensemblⁱ	ENST00000253801; ENSP00000253801; ENSG00000131482 [P35575-1] ENST00000592383; ENSP00000465958; ENSG00000131482 [P35575-2]
GeneIDⁱ	2538
KEGGⁱ	hsa:2538
UCSC genome browser More...UCSCⁱ	uc002icb.3 human [P35575-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P35575	Glucose-6-phosphatase, catalytic (Glycogen storage disease type I, von Gierke disease), isoform CRA_a		HUMAN		359	UniRef100_P35575

Protein	Similar proteins		Species	Score	Length	Source
P35575	Glucose-6-phosphatase		BOVIN		357	UniRef90_P35575
Glucose-6-phosphatase		CANLF		357
Glucose-6-phosphatase		FELCA		357
Glucose-6-phosphatase, catalytic (Glycogen storage disease type I, von Gierke disease), isoform CRA_a		HUMAN		359
glucose-6-phosphatase-like		BALAS		366
+99
P35575-2	G6PC isoform 3		PONAB		176	UniRef90_P35575-2
G6PC isoform 3		PANTR		176
glucose-6-phosphatase isoform X2		PHYCD		166
UPI0004F01FD7		CALJA		178
UPI0004F0C0D4		PANPA		178
+7

Protein	Similar proteins		Species	Score	Length	Source
P35575	Glucose-6-phosphatase		BOVIN		357	UniRef50_P35575
Glucose-6-phosphatase		FELCA		357
Glucose-6-phosphatase		CANLF		357
Glucose-6-phosphatase, catalytic (Glycogen storage disease type I, von Gierke disease), isoform CRA_a		HUMAN		359
glucose-6-phosphatase-like		BALAS		366
+479
P35575-2	G6PC isoform 3		PANTR		176	UniRef50_P35575-2
G6PC isoform 3		PONAB		176
glucose-6-phosphatase isoform X2		PHYCD		166
Uncharacterized protein		PROCO		199
UPI0004F01FD7		CALJA		178
+10

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	U01120 mRNA Translation: AAA16222.1 AK303771 mRNA Translation: BAG64735.1 Different initiation. AK313982 mRNA Translation: BAG36695.1 AC016889 Genomic DNA No translation available. BC130478 mRNA Translation: AAI30479.1 BC136369 mRNA Translation: AAI36370.1
CCDSⁱ	CCDS11446.1 [P35575-1] CCDS59291.1 [P35575-2]
PIRⁱ	A48251
RefSeqⁱ	NP_000142.2, NM_000151.3 [P35575-1] NP_001257326.1, NM_001270397.1 [P35575-2]
UniGeneⁱ	Hs.212293 Hs.742566

3D structure databases

ProteinModelPortalⁱ	P35575
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	108813, 3 interactors
IntActⁱ	P35575, 3 interactors
STRINGⁱ	9606.ENSP00000253801

Chemistry databases

BindingDBⁱ	P35575
ChEMBLⁱ	CHEMBL2282

PTM databases

DEPODⁱ	P35575
GlyConnectⁱ	1267
iPTMnetⁱ	P35575
PhosphoSitePlusⁱ	P35575

Polymorphism and mutation databases

BioMutaⁱ	G6PC
DMDMⁱ	206729864

Proteomic databases

PaxDbⁱ	P35575
PeptideAtlasⁱ	P35575
PRIDEⁱ	P35575
ProteomicsDBⁱ	55092

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	2538
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000253801; ENSP00000253801; ENSG00000131482 [P35575-1] ENST00000592383; ENSP00000465958; ENSG00000131482 [P35575-2]
GeneIDⁱ	2538
KEGGⁱ	hsa:2538
UCSCⁱ	uc002icb.3 human [P35575-1]

Organism-specific databases

CTDⁱ	2538
DisGeNETⁱ	2538
EuPathDBⁱ	HostDB:ENSG00000131482.9
GeneCardsⁱ	G6PC
GeneReviewsⁱ	G6PC
HGNCⁱ	HGNC:4056 G6PC
HPAⁱ	HPA052324
MalaCardsⁱ	G6PC
MIMⁱ	232200 phenotype 613742 gene
neXtProtⁱ	NX_P35575
OpenTargetsⁱ	ENSG00000131482
Orphanetⁱ	79258 Glycogen storage disease due to glucose-6-phosphatase deficiency type Ia
PharmGKBⁱ	PA28468
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	ENOG410IDXG Eukaryota ENOG4110AJ7 LUCA
GeneTreeⁱ	ENSGT00510000046465
HOGENOMⁱ	HOG000264239
HOVERGENⁱ	HBG003560
InParanoidⁱ	P35575
KOⁱ	K01084
OMAⁱ	RYLQVNY
OrthoDBⁱ	EOG091G0AXF
PhylomeDBⁱ	P35575
TreeFamⁱ	TF324388

Enzyme and pathway databases

UniPathwayⁱ	UPA00138
BioCycⁱ	MetaCyc:HS05538-MONOMER
BRENDAⁱ	3.1.3.9 2681
Reactomeⁱ	R-HSA-3274531 Glycogen storage disease type Ia (G6PC) R-HSA-70263 Gluconeogenesis
SABIO-RKⁱ	P35575
SIGNORⁱ	P35575

Miscellaneous databases

ChiTaRSⁱ	G6PC human
GeneWikiⁱ	G6PC
GenomeRNAiⁱ	2538
Protein Ontology More...PROⁱ	PR:P35575
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000131482 Expressed in 58 organ(s), highest expression level in liver
CleanExⁱ	HS_G6PC
ExpressionAtlasⁱ	P35575 baseline and differential
Genevisibleⁱ	P35575 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR016275 Glucose-6-phosphatase IPR036938 P_Acid_Pase_2/haloperoxi_sf IPR000326 P_Acid_Pase_2/haloperoxidase
Pfamⁱ	View protein in Pfam PF01569 PAP2, 1 hit
PIRSFⁱ	PIRSF000905 Glucose-6-phosphatase, 1 hit
SMARTⁱ	View protein in SMART SM00014 acidPPc, 1 hit
SUPFAMⁱ	SSF48317 SSF48317, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	G6PC_HUMAN
Accessionⁱ	P35575Primary (citable) accession number: P35575 Secondary accession number(s): A1L4C0, B4E1C3, K7EL82
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	June 1, 1994
	Last sequence update:	September 23, 2008
	Last modified:	November 7, 2018
	This is version 175 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
PATHWAY comments
Index of metabolic and biosynthesis pathways
Human chromosome 17
Human chromosome 17: entries, gene names and cross-references to MIM

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - P35575 (G6PC_HUMAN)

Your basket is currently empty. i <p>When browsing through different UniProt proteins, you can use the ‘basket’ to save them, so that you can back to find or analyse them later.<p><a href='/help/basket' target='_top'>More...</a></p>

Glucose-6-phosphatase

G6PC

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: gluconeogenesis

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Endoplasmic reticulum

Endoplasmic reticulum

Other locations

Topology

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Mutagenesis

Keywords - Diseasei

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

Chemistry databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

Experimental Info

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

UniProt is an ELIXIR core data resource

Your basket is currently empty. ⁱ

Functionⁱ

Pathwayⁱ: gluconeogenesis

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Sequence cautionⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ