Seroatlas · Human Serome Atlas

PCK2

Phosphoenolpyruvate carboxykinase [GTP], mitochondrial

Also known as: PCKGM_HUMAN, PEPCK, PEPCK2

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
Q16822
Gene
PCK2
Ensembl
ENSG00000100889
Chromosome
14
Canonical length
640 aa
Protein class
Citric acid cycle related proteins, Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins
Subcellular location
Mitochondria

OverviewNCBI Gene

This gene encodes a mitochondrial enzyme that catalyzes the conversion of oxaloacetate to phosphoenolpyruvate in the presence of guanosine triphosphate (GTP). A cytosolic form of this protein is encoded by a different gene and is the key enzyme of gluconeogenesis in the liver. Alternatively spliced transcript variants have been described. [provided by RefSeq, Apr 2014]

Canonical amino-acid sequenceUniProt

640 residues, UniProt reviewed canonical sequence.

>Q16822|PCK2
     1  MAALYRPGLR LNWHGLSPLG WPSCRSIQTL RVLSGDLGQL PTGIRDFVEH SARLCQPEGI
    61  HICDGTEAEN TATLTLLEQQ GLIRKLPKYN NCWLARTDPK DVARVESKTV IVTPSQRDTV
   121  PLPPGGARGQ LGNWMSPADF QRAVDERFPG CMQGRTMYVL PFSMGPVGSP LSRIGVQLTD
   181  SAYVVASMRI MTRLGTPVLQ ALGDGDFVKC LHSVGQPLTG QGEPVSQWPC NPEKTLIGHV
   241  PDQREIISFG SGYGGNSLLG KKCFALRIAS RLARDEGWLA EHMLILGITS PAGKKRYVAA
   301  AFPSACGKTN LAMMRPALPG WKVECVGDDI AWMRFDSEGR LRAINPENGF FGVAPGTSAT
   361  TNPNAMATIQ SNTIFTNVAE TSDGGVYWEG IDQPLPPGVT VTSWLGKPWK PGDKEPCAHP
   421  NSRFCAPARQ CPIMDPAWEA PEGVPIDAII FGGRRPKGVP LVYEAFNWRH GVFVGSAMRS
   481  ESTAAAEHKG KIIMHDPFAM RPFFGYNFGH YLEHWLSMEG RKGAQLPRIF HVNWFRRDEA
   541  GHFLWPGFGE NARVLDWICR RLEGEDSARE TPIGLVPKEG ALDLSGLRAI DTTQLFSLPK
   601  DFWEQEVRDI RSYLTEQVNQ DLPKEVLAEL EALERRVHKM

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against PCK2 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Intracellular
Secreted
No
Transmembrane segments
0
Mean surface accessibility (rSASA)
0.23
Highest tissue expression
367 nTPM

Expression across tissuesHPA

Tissue

  • liver: 367 nTPM
  • duodenum: 185 nTPM
  • small intestine: 128 nTPM
  • kidney: 93 nTPM
  • colon: 45 nTPM
  • pancreas: 34 nTPM

Single-cell type

  • enterocytes: 41 nCPM
  • proximal tubule cells: 41 nCPM
  • hepatocytes: 14 nCPM
  • late primary spermatocytes: 12 nCPM
  • early spermatids: 12 nCPM
  • renal collecting duct principal cells: 8.7 nCPM

Immune cell

  • non-classical monocyte: 22 nTPM
  • intermediate monocyte: 22 nTPM
  • myeloid DC: 15 nTPM
  • classical monocyte: 12 nTPM
  • memory B-cell: 11 nTPM
  • T-reg: 9.1 nTPM

Brain region

  • medulla oblongata: 7.8 nTPM
  • midbrain: 7.2 nTPM
  • white matter: 6.8 nTPM
  • hypothalamus: 6.7 nTPM
  • spinal cord: 6.7 nTPM
  • pons: 6.6 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about PCK2.

Disease | AllUniProt

Conditions PCK2 is implicated in, by any mechanism.

  • Mitochondrial phosphoenolpyruvate carboxykinase deficiency (M-PEPCKD) MIM:261650

Disease | GeneticClinVar

4 pathogenic / likely-pathogenic of 325 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

  • Phosphoenolpyruvate carboxykinase deficiency, mitochondrial

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)
1.39
gnomAD pLI
0
gnomAD missense Z
-0.2
DepMap mean gene effect
-0.04
DepMap dependency class
selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of PCK2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads PCK2 as an antibody target. Whether an autoantibody or antibody against PCK2 could matter depends on whether native PCK2 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

PCK2 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label PCK2 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/PCK2. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

© 2026 · Seroatlas, Discover Oregon LLC · Terms of use

Loading the interactive Seroatlas protein explorer...