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Protein

Phosphoenolpyruvate carboxykinase, cytosolic [GTP]

Gene

PCK1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<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

Regulates cataplerosis and anaplerosis, the processes that control the levels of metabolic intermediates in the citric acid cycle. At low glucose levels, it catalyzes the cataplerotic conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle. At high glucose levels, it catalyzes the anaplerotic conversion of phosphoenolpyruvate to oxaloacetate.4 Publications

Miscellaneous

In eukaryotes there are two isozymes: a cytoplasmic one and a mitochondrial one.

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Mn2+3 PublicationsNote: Binds 1 Mn2+ ion per subunit.3 Publications

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Enzyme activity is regulated by acetylation and glucose levels (PubMed:30193097, PubMed:20167786). The anaplerotic conversion of phosphoenolpyruvate to oxaloacetate is improved by PCK1 acetylation on Lys-91 (K91ac), Lys-473 (K473ac) and Lys-521 (K521ac) (By similarity). High glucose concentrations favor PCK1 anaplerotic activity by triggering acetylation on Lys-91 (K91ac). At low glucose levels, SIRT1-mediated deacetylation of Lys-91 promotes the cataplerotic conversion of oxaloacetate to phosphoenolpyruvate (PubMed:30193097).By similarity2 Publications

<p>This subsection of the ‘Function’ section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

Kcat is 33 sec(-1) with oxaloacetate as substrate (for the enzyme purified from cells exposed to 10mM glucose). Kcat is 21 sec(-1) with oxaloacetate as substrate (for the enzyme purified from cells exposed to 15mM glucose). Kcat is 17 sec(-1) with phosphoenolpyruvate as substrate (for the enzyme purified from cells exposed to 10mM glucose). Kcat is 11 sec(-1)with phosphoenolpyruvate as substrate (for the enzyme purified from cells exposed to 15mM glucose). Kcat is 40 sec(-1) with GTP as substrate (for the enzyme purified from cells exposed to 10mM glucose). Kcat is 22 sec(-1) with GTP as substrate (for the enzyme purified from cells exposed to 15mM glucose). Kcat is 16 sec(-1) with GDP as substrate (for the enzyme purified from cells exposed to 10mM glucose). Kcat is 11 sec(-1) with GDP as substrate (for the enzyme purified from cells exposed to 15mM glucose).1 Publication
  1. KM=35 µM for oxaloacetate (for the enzyme purified from cells exposed to 10mM glucose)1 Publication
  2. KM=46 µM for oxaloacetate (for the enzyme purified from cells exposed to 15mM glucose)1 Publication
  3. KM=170 µM for GTP (for the enzyme purified from cells exposed to 10mM glucose)1 Publication
  4. KM=151 µM for GTP (for the enzyme purified from cells exposed to 15mM glucose)1 Publication
  5. KM=208 µM for phosphoenolpyruvate (for the enzyme purified from cells exposed to 10mM glucose)1 Publication
  6. KM=87 µM for phosphoenolpyruvate (for the enzyme purified from cells exposed to 15mM glucose)1 Publication
  7. KM=63 µM for GDP (for the enzyme purified from cells exposed to 10mM glucose)1 Publication
  8. KM=29 µM for GDP (for the enzyme purified from cells exposed to 15mM glucose)1 Publication

    <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

    This protein is involved in the pathway gluconeogenesis, which is part of Carbohydrate biosynthesis.4 Publications
    View all proteins of this organism that are known to be involved in the pathway gluconeogenesis and in Carbohydrate biosynthesis.

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei87Substrate3 Publications1
    <p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi244Manganese3 Publications1
    Metal bindingi264Manganese; via tele nitrogen3 Publications1
    Binding sitei286SubstrateBy similarity1
    <p>This subsection of the ‘Function’ section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei2881 Publication1
    Metal bindingi311Manganese3 Publications1
    Binding sitei405GTP1 Publication1
    Binding sitei436GTP1 Publication1

    Regions

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Function’ section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi287 – 292GTP1 Publication6
    Nucleotide bindingi530 – 533GTP3 Publications4

    <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

    <p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

    Molecular functionDecarboxylase, Lyase
    Biological processGluconeogenesis
    LigandGTP-binding, Manganese, Metal-binding, Nucleotide-binding

    Enzyme and pathway databases

    BioCyc Collection of Pathway/Genome Databases

    More...
    BioCyci
    MetaCyc:HS04751-MONOMER

    BRENDA Comprehensive Enzyme Information System

    More...
    BRENDAi
    4.1.1.32 2681

    Reactome - a knowledgebase of biological pathways and processes

    More...
    Reactomei
    R-HSA-2161541 Abacavir metabolism
    R-HSA-381340 Transcriptional regulation of white adipocyte differentiation
    R-HSA-70263 Gluconeogenesis

    SABIO-RK: Biochemical Reaction Kinetics Database

    More...
    SABIO-RKi
    P35558

    SIGNOR Signaling Network Open Resource

    More...
    SIGNORi
    P35558

    UniPathway: a resource for the exploration and annotation of metabolic pathways

    More...
    UniPathwayi
    UPA00138

    <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

    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
    Recommended name:
    Phosphoenolpyruvate carboxykinase, cytosolic [GTP] (EC:4.1.1.324 Publications)
    Short name:
    PEPCK-C
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
    Name:PCK1Imported
    Synonyms:PEPCK1
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
    • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 20

    Organism-specific databases

    Eukaryotic Pathogen Database Resources

    More...
    EuPathDBi
    HostDB:ENSG00000124253.10

    Human Gene Nomenclature Database

    More...
    HGNCi
    HGNC:8724 PCK1

    Online Mendelian Inheritance in Man (OMIM)

    More...
    MIMi
    614168 gene

    neXtProt; the human protein knowledge platform

    More...
    neXtProti
    NX_P35558

    <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

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Cytoplasm

    <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

    <p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

    Phosphoenolpyruvate carboxykinase deficiency, cytosolic (PCKDC)3 Publications
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionAn autosomal recessive metabolic disorder characterized by impaired gluconeogenesis, hypoglycemia, hypotonia, hepatomegaly, hepatic dysfunction, failure to thrive, lactic acidosis, and elevated tricarboxylic acid intermediates, particularly fumarate, in urine.
    See also OMIM:261680
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_07963345I → T in PCKDC; decreased stability; no effect on phosphoenolpyruvate carboxykinase activity. 1 PublicationCorresponds to variant dbSNP:rs202197769EnsemblClinVar.1
    Natural variantiVAR_079634309G → R in PCKDC; unknown pathological significance; decreased phosphoenolpyruvate carboxykinase activity. 1 PublicationCorresponds to variant dbSNP:rs201186470EnsemblClinVar.1
    Natural variantiVAR_079635440 – 443Missing in PCKDC; decreased phosphoenolpyruvate carboxykinase activity. 1 Publication4

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi70K → R: Abolishes acetylation and increases protein stability; when associated with R-71 and R-594. 1 Publication1
    Mutagenesisi71K → R: Abolishes acetylation and increases protein stability; when associated with R-70 and R-594. 1 Publication1
    Mutagenesisi594K → R: Abolishes acetylation and increases protein stability; when associated with R-70 and R-71. 1 Publication1

    Organism-specific databases

    DisGeNET

    More...
    DisGeNETi
    5105

    MalaCards human disease database

    More...
    MalaCardsi
    PCK1
    MIMi261680 phenotype

    Open Targets

    More...
    OpenTargetsi
    ENSG00000124253

    Orphanet; a database dedicated to information on rare diseases and orphan drugs

    More...
    Orphaneti
    2880 Phosphoenolpyruvate carboxykinase deficiency

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...
    PharmGKBi
    PA33069

    Chemistry databases

    ChEMBL database of bioactive drug-like small molecules

    More...
    ChEMBLi
    CHEMBL2911

    Drug and drug target database

    More...
    DrugBanki
    DB02008 1-(2-Fluorobenzyl)-3-Butyl-8-(N-Acetyl-4-Aminobenzyl)-Xanthine
    DB03267 1-Allyl-3-Butyl-8-(N-Acetyl-4-Aminobenzyl)-Xanthine
    DB01819 Phosphoenolpyruvate
    DB03725 Phosphomethylphosphonic Acid-Guanylate Ester

    Polymorphism and mutation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...
    BioMutai
    PCK1

    Domain mapping of disease mutations (DMDM)

    More...
    DMDMi
    93138710

    <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

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001036271 – 622Phosphoenolpyruvate carboxykinase, cytosolic [GTP]Add BLAST622

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei19PhosphoserineCombined sources1
    Modified residuei70N6-acetyllysine; by p300/EP3002 Publications1
    Modified residuei71N6-acetyllysine; by p300/EP3002 Publications1
    Modified residuei91N6-acetyllysine; by p300/EP3001 Publication1
    Modified residuei118PhosphoserineBy similarity1
    Modified residuei178PhosphothreonineBy similarity1
    Modified residuei286PhosphoserineBy similarity1
    Modified residuei473N6-acetyllysineBy similarity1
    Modified residuei521N6-acetyllysineBy similarity1
    Modified residuei524N6-acetyllysineBy similarity1
    Modified residuei594N6-acetyllysine; by p300/EP3002 Publications1

    <p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

    Acetylated. Lysine acetylation by p300/EP300 is increased on high glucose conditions (PubMed:20167786, PubMed:21726808, PubMed:30193097). Lysine acetylation promotes ubiquitination by UBR5 (PubMed:21726808). Acetylation is enhanced in the presence of BAG6. Deacetylated by SIRT2. Deacetylation of Lys-91 is carried out by SIRT1 and depends on PCK1 phosphorylation levels (PubMed:30193097).3 Publications
    Phosphorylated in a GSK3B-mediated pathway; phosphorylation affects the efficiency of SIRT1-mediated deacetylation, and regulates PCK1 ubiquitination and degradation.1 Publication
    Ubiquitination by UBR5 leads to proteasomal degradation.1 Publication

    Keywords - PTMi

    Acetylation, Phosphoprotein, Ubl conjugation

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...
    EPDi
    P35558

    jPOST - Japan Proteome Standard Repository/Database

    More...
    jPOSTi
    P35558

    MaxQB - The MaxQuant DataBase

    More...
    MaxQBi
    P35558

    PaxDb, a database of protein abundance averages across all three domains of life

    More...
    PaxDbi
    P35558

    PeptideAtlas

    More...
    PeptideAtlasi
    P35558

    PRoteomics IDEntifications database

    More...
    PRIDEi
    P35558

    ProteomicsDB human proteome resource

    More...
    ProteomicsDBi
    55087

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...
    iPTMneti
    P35558

    Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

    More...
    PhosphoSitePlusi
    P35558

    <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

    <p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

    Major sites of expression are liver, kidney and adipocytes.

    <p>This subsection of the ‘Expression’ section reports the experimentally proven effects of inducers and repressors (usually chemical compounds or environmental factors) on the level of protein (or mRNA) expression (up-regulation, down-regulation, constitutive expression).<p><a href='/help/induction' target='_top'>More...</a></p>Inductioni

    Regulated by cAMP and insulin.1 Publication

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...
    Bgeei
    ENSG00000124253 Expressed in 157 organ(s), highest expression level in cortex of kidney

    CleanEx database of gene expression profiles

    More...
    CleanExi
    HS_PCK1

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...
    Genevisiblei
    P35558 HS

    Organism-specific databases

    Human Protein Atlas

    More...
    HPAi
    CAB017027
    HPA006277
    HPA006507

    <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

    <p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Monomer.2 Publications

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGrid)

    More...
    BioGridi
    111136, 78 interactors

    Protein interaction database and analysis system

    More...
    IntActi
    P35558, 2 interactors

    Molecular INTeraction database

    More...
    MINTi
    P35558

    STRING: functional protein association networks

    More...
    STRINGi
    9606.ENSP00000319814

    Chemistry databases

    BindingDB database of measured binding affinities

    More...
    BindingDBi
    P35558

    <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

    Secondary structure

    1622
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    Select the link destinations:

    Protein Data Bank Europe

    More...
    PDBei

    Protein Data Bank RCSB

    More...
    RCSB PDBi

    Protein Data Bank Japan

    More...
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    1KHBX-ray1.85A1-622[»]
    1KHEX-ray2.40A1-622[»]
    1KHFX-ray2.02A1-622[»]
    1KHGX-ray2.34A1-622[»]
    1M51X-ray2.25A1-622[»]
    1NHXX-ray2.10A1-622[»]
    2GMVX-ray2.30A/B1-622[»]

    Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

    More...
    ProteinModelPortali
    P35558

    SWISS-MODEL Repository - a database of annotated 3D protein structure models

    More...
    SMRi
    P35558

    Database of comparative protein structure models

    More...
    ModBasei
    Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...
    MobiDBi
    Search...

    Miscellaneous databases

    Relative evolutionary importance of amino acids within a protein sequence

    More...
    EvolutionaryTracei
    P35558

    <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

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni235 – 237Substrate binding3 Publications3
    Regioni403 – 405Substrate binding3 Publications3

    <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

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...
    eggNOGi
    KOG3749 Eukaryota
    COG1274 LUCA

    Ensembl GeneTree

    More...
    GeneTreei
    ENSGT00390000001912

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...
    HOGENOMi
    HOG000191700

    The HOVERGEN Database of Homologous Vertebrate Genes

    More...
    HOVERGENi
    HBG053651

    InParanoid: Eukaryotic Ortholog Groups

    More...
    InParanoidi
    P35558

    KEGG Orthology (KO)

    More...
    KOi
    K01596

    Identification of Orthologs from Complete Genome Data

    More...
    OMAi
    GPTNNWV

    Database of Orthologous Groups

    More...
    OrthoDBi
    286671at2759

    Database for complete collections of gene phylogenies

    More...
    PhylomeDBi
    P35558

    TreeFam database of animal gene trees

    More...
    TreeFami
    TF314402

    Family and domain databases

    Conserved Domains Database

    More...
    CDDi
    cd00819 PEPCK_GTP, 1 hit

    Gene3D Structural and Functional Annotation of Protein Families

    More...
    Gene3Di
    3.40.449.10, 1 hit
    3.90.228.20, 1 hit

    HAMAP database of protein families

    More...
    HAMAPi
    MF_00452 PEPCK_GTP, 1 hit

    Integrated resource of protein families, domains and functional sites

    More...
    InterProi
    View protein in InterPro
    IPR018091 PEP_carboxykin_GTP_CS
    IPR013035 PEP_carboxykinase_C
    IPR008209 PEP_carboxykinase_GTP
    IPR035077 PEP_carboxykinase_GTP_C
    IPR035078 PEP_carboxykinase_GTP_N
    IPR008210 PEP_carboxykinase_N

    The PANTHER Classification System

    More...
    PANTHERi
    PTHR11561 PTHR11561, 1 hit

    Pfam protein domain database

    More...
    Pfami
    View protein in Pfam
    PF00821 PEPCK_GTP, 1 hit
    PF17297 PEPCK_N, 1 hit

    PIRSF; a whole-protein classification database

    More...
    PIRSFi
    PIRSF001348 PEP_carboxykinase_GTP, 1 hit

    Superfamily database of structural and functional annotation

    More...
    SUPFAMi
    SSF68923 SSF68923, 1 hit

    PROSITE; a protein domain and family database

    More...
    PROSITEi
    View protein in PROSITE
    PS00505 PEPCK_GTP, 1 hit

    <p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2)i

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

    This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket
    Isoform 1 (identifier: P35558-1) [UniParc]FASTAAdd 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
    MPPQLQNGLN LSAKVVQGSL DSLPQAVREF LENNAELCQP DHIHICDGSE
    60 70 80 90 100
    EENGRLLGQM EEEGILRRLK KYDNCWLALT DPRDVARIES KTVIVTQEQR
    110 120 130 140 150
    DTVPIPKTGL SQLGRWMSEE DFEKAFNARF PGCMKGRTMY VIPFSMGPLG
    160 170 180 190 200
    SPLSKIGIEL TDSPYVVASM RIMTRMGTPV LEAVGDGEFV KCLHSVGCPL
    210 220 230 240 250
    PLQKPLVNNW PCNPELTLIA HLPDRREIIS FGSGYGGNSL LGKKCFALRM
    260 270 280 290 300
    ASRLAKEEGW LAEHMLILGI TNPEGEKKYL AAAFPSACGK TNLAMMNPSL
    310 320 330 340 350
    PGWKVECVGD DIAWMKFDAQ GHLRAINPEN GFFGVAPGTS VKTNPNAIKT
    360 370 380 390 400
    IQKNTIFTNV AETSDGGVYW EGIDEPLASG VTITSWKNKE WSSEDGEPCA
    410 420 430 440 450
    HPNSRFCTPA SQCPIIDAAW ESPEGVPIEG IIFGGRRPAG VPLVYEALSW
    460 470 480 490 500
    QHGVFVGAAM RSEATAAAEH KGKIIMHDPF AMRPFFGYNF GKYLAHWLSM
    510 520 530 540 550
    AQHPAAKLPK IFHVNWFRKD KEGKFLWPGF GENSRVLEWM FNRIDGKAST
    560 570 580 590 600
    KLTPIGYIPK EDALNLKGLG HINMMELFSI SKEFWEKEVE DIEKYLEDQV
    610 620
    NADLPCEIER EILALKQRIS QM
    Length:622
    Mass (Da):69,195
    Last modified:March 7, 2006 - v3
    <p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i78D309E0845CC181
    GO
    Isoform 2 (identifier: P35558-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-3: MPP → MTT
         4-320: Missing.

    Note: No experimental confirmation available.
    Show »
    Length:305
    Mass (Da):34,156
    Checksum:i2FDE6275A0F15D6C
    GO

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti250M → N in AAA02558 (PubMed:8325643).Curated1
    Sequence conflicti256K → E in AAA60084 (PubMed:8490617).Curated1
    Sequence conflicti291T → S in AAA02558 (PubMed:8325643).Curated1
    Sequence conflicti551 – 552KL → NV in AAA60084 (PubMed:8490617).Curated2
    Sequence conflicti597E → V in AAA60084 (PubMed:8490617).Curated1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_07963345I → T in PCKDC; decreased stability; no effect on phosphoenolpyruvate carboxykinase activity. 1 PublicationCorresponds to variant dbSNP:rs202197769EnsemblClinVar.1
    Natural variantiVAR_02107255R → Q1 PublicationCorresponds to variant dbSNP:rs28383585Ensembl.1
    Natural variantiVAR_02107360M → T1 PublicationCorresponds to variant dbSNP:rs28383586EnsemblClinVar.1
    Natural variantiVAR_021074138T → I1 PublicationCorresponds to variant dbSNP:rs28359542Ensembl.1
    Natural variantiVAR_021075184V → L5 PublicationsCorresponds to variant dbSNP:rs707555EnsemblClinVar.1
    Natural variantiVAR_015575267I → V2 PublicationsCorresponds to variant dbSNP:rs8192708EnsemblClinVar.1
    Natural variantiVAR_021076276E → K1 PublicationCorresponds to variant dbSNP:rs11552145EnsemblClinVar.1
    Natural variantiVAR_079634309G → R in PCKDC; unknown pathological significance; decreased phosphoenolpyruvate carboxykinase activity. 1 PublicationCorresponds to variant dbSNP:rs201186470EnsemblClinVar.1
    Natural variantiVAR_021077368V → I1 PublicationCorresponds to variant dbSNP:rs1804160EnsemblClinVar.1
    Natural variantiVAR_021078427P → S1 PublicationCorresponds to variant dbSNP:rs28359550Ensembl.1
    Natural variantiVAR_079635440 – 443Missing in PCKDC; decreased phosphoenolpyruvate carboxykinase activity. 1 Publication4
    Natural variantiVAR_042444586E → D1 PublicationCorresponds to variant dbSNP:rs1042529Ensembl.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0570731 – 3MPP → MTT in isoform 2. 1 Publication3
    Alternative sequenceiVSP_0570744 – 320Missing in isoform 2. 1 PublicationAdd BLAST317

    Sequence databases

    Select the link destinations:

    EMBL nucleotide sequence database

    More...
    EMBLi

    GenBank nucleotide sequence database

    More...
    GenBanki

    DNA Data Bank of Japan; a nucleotide sequence database

    More...
    DDBJi
    Links Updated
    L05144 mRNA Translation: AAA60084.1
    L12760 Genomic DNA Translation: AAA02558.1
    AY794987 Genomic DNA Translation: AAV50001.1
    AK290802 mRNA Translation: BAF83491.1
    AK300072 mRNA Translation: BAG61876.1
    AL035541 Genomic DNA No translation available.
    CH471077 Genomic DNA Translation: EAW75514.1
    BC023978 mRNA Translation: AAH23978.1
    U31519 Genomic DNA Translation: AAA91026.1

    The Consensus CDS (CCDS) project

    More...
    CCDSi
    CCDS13460.1 [P35558-1]

    Protein sequence database of the Protein Information Resource

    More...
    PIRi
    A45746

    NCBI Reference Sequences

    More...
    RefSeqi
    NP_002582.3, NM_002591.3 [P35558-1]

    UniGene gene-oriented nucleotide sequence clusters

    More...
    UniGenei
    Hs.1872

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...
    Ensembli
    ENST00000319441; ENSP00000319814; ENSG00000124253 [P35558-1]

    Database of genes from NCBI RefSeq genomes

    More...
    GeneIDi
    5105

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...
    KEGGi
    hsa:5105

    UCSC genome browser

    More...
    UCSCi
    uc002xyn.5 human [P35558-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    <p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

    <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

    <p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

    NIEHS-SNPs

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    L05144 mRNA Translation: AAA60084.1
    L12760 Genomic DNA Translation: AAA02558.1
    AY794987 Genomic DNA Translation: AAV50001.1
    AK290802 mRNA Translation: BAF83491.1
    AK300072 mRNA Translation: BAG61876.1
    AL035541 Genomic DNA No translation available.
    CH471077 Genomic DNA Translation: EAW75514.1
    BC023978 mRNA Translation: AAH23978.1
    U31519 Genomic DNA Translation: AAA91026.1
    CCDSiCCDS13460.1 [P35558-1]
    PIRiA45746
    RefSeqiNP_002582.3, NM_002591.3 [P35558-1]
    UniGeneiHs.1872

    3D structure databases

    Select the link destinations:
    PDBei
    RCSB PDBi
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    1KHBX-ray1.85A1-622[»]
    1KHEX-ray2.40A1-622[»]
    1KHFX-ray2.02A1-622[»]
    1KHGX-ray2.34A1-622[»]
    1M51X-ray2.25A1-622[»]
    1NHXX-ray2.10A1-622[»]
    2GMVX-ray2.30A/B1-622[»]
    ProteinModelPortaliP35558
    SMRiP35558
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi111136, 78 interactors
    IntActiP35558, 2 interactors
    MINTiP35558
    STRINGi9606.ENSP00000319814

    Chemistry databases

    BindingDBiP35558
    ChEMBLiCHEMBL2911
    DrugBankiDB02008 1-(2-Fluorobenzyl)-3-Butyl-8-(N-Acetyl-4-Aminobenzyl)-Xanthine
    DB03267 1-Allyl-3-Butyl-8-(N-Acetyl-4-Aminobenzyl)-Xanthine
    DB01819 Phosphoenolpyruvate
    DB03725 Phosphomethylphosphonic Acid-Guanylate Ester

    PTM databases

    iPTMnetiP35558
    PhosphoSitePlusiP35558

    Polymorphism and mutation databases

    BioMutaiPCK1
    DMDMi93138710

    Proteomic databases

    EPDiP35558
    jPOSTiP35558
    MaxQBiP35558
    PaxDbiP35558
    PeptideAtlasiP35558
    PRIDEiP35558
    ProteomicsDBi55087

    Protocols and materials databases

    The DNASU plasmid repository

    More...
    DNASUi
    5105
    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENST00000319441; ENSP00000319814; ENSG00000124253 [P35558-1]
    GeneIDi5105
    KEGGihsa:5105
    UCSCiuc002xyn.5 human [P35558-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...
    CTDi
    5105
    DisGeNETi5105
    EuPathDBiHostDB:ENSG00000124253.10

    GeneCards: human genes, protein and diseases

    More...
    GeneCardsi
    PCK1

    H-Invitational Database, human transcriptome db

    More...
    H-InvDBi
    HIX0015941
    HGNCiHGNC:8724 PCK1
    HPAiCAB017027
    HPA006277
    HPA006507
    MalaCardsiPCK1
    MIMi261680 phenotype
    614168 gene
    neXtProtiNX_P35558
    OpenTargetsiENSG00000124253
    Orphaneti2880 Phosphoenolpyruvate carboxykinase deficiency
    PharmGKBiPA33069

    GenAtlas: human gene database

    More...
    GenAtlasi
    Search...

    Phylogenomic databases

    eggNOGiKOG3749 Eukaryota
    COG1274 LUCA
    GeneTreeiENSGT00390000001912
    HOGENOMiHOG000191700
    HOVERGENiHBG053651
    InParanoidiP35558
    KOiK01596
    OMAiGPTNNWV
    OrthoDBi286671at2759
    PhylomeDBiP35558
    TreeFamiTF314402

    Enzyme and pathway databases

    UniPathwayi
    UPA00138

    BioCyciMetaCyc:HS04751-MONOMER
    BRENDAi4.1.1.32 2681
    ReactomeiR-HSA-2161541 Abacavir metabolism
    R-HSA-381340 Transcriptional regulation of white adipocyte differentiation
    R-HSA-70263 Gluconeogenesis
    SABIO-RKiP35558
    SIGNORiP35558

    Miscellaneous databases

    ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

    More...
    ChiTaRSi
    PCK1 human
    EvolutionaryTraceiP35558

    The Gene Wiki collection of pages on human genes and proteins

    More...
    GeneWikii
    PCK1

    Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

    More...
    GenomeRNAii
    5105

    Protein Ontology

    More...
    PROi
    PR:P35558

    The Stanford Online Universal Resource for Clones and ESTs

    More...
    SOURCEi
    Search...

    Gene expression databases

    BgeeiENSG00000124253 Expressed in 157 organ(s), highest expression level in cortex of kidney
    CleanExiHS_PCK1
    GenevisibleiP35558 HS

    Family and domain databases

    CDDicd00819 PEPCK_GTP, 1 hit
    Gene3Di3.40.449.10, 1 hit
    3.90.228.20, 1 hit
    HAMAPiMF_00452 PEPCK_GTP, 1 hit
    InterProiView protein in InterPro
    IPR018091 PEP_carboxykin_GTP_CS
    IPR013035 PEP_carboxykinase_C
    IPR008209 PEP_carboxykinase_GTP
    IPR035077 PEP_carboxykinase_GTP_C
    IPR035078 PEP_carboxykinase_GTP_N
    IPR008210 PEP_carboxykinase_N
    PANTHERiPTHR11561 PTHR11561, 1 hit
    PfamiView protein in Pfam
    PF00821 PEPCK_GTP, 1 hit
    PF17297 PEPCK_N, 1 hit
    PIRSFiPIRSF001348 PEP_carboxykinase_GTP, 1 hit
    SUPFAMiSSF68923 SSF68923, 1 hit
    PROSITEiView protein in PROSITE
    PS00505 PEPCK_GTP, 1 hit

    ProtoNet; Automatic hierarchical classification of proteins

    More...
    ProtoNeti
    Search...

    <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 subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiPCKGC_HUMAN
    <p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P35558
    Secondary accession number(s): A8K437
    , B4DT64, Q8TCA3, Q9UJD2
    <p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
    Last sequence update: March 7, 2006
    Last modified: January 16, 2019
    This is version 195 of the entry and version 3 of the sequence. See complete history.
    <p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny 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.

    <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

    3D-structure, Complete proteome, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    7. Human chromosome 20
      Human chromosome 20: entries, gene names and cross-references to MIM
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