Seroatlas · Human Serome Atlas

PDHB

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

Also known as: E1beta, ODPB_HUMAN, PDHE1B

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

Protein identityUniProt · HPA

UniProt accession
P11177
Gene
PDHB
Ensembl
ENSG00000168291
Chromosome
3
Canonical length
359 aa
Protein class
Citric acid cycle related proteins, Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted intracellular proteins
Subcellular location
Nucleoplasm,Mitochondria

OverviewNCBI Gene

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]

Canonical amino-acid sequenceUniProt

359 residues, UniProt reviewed canonical sequence.

>P11177|PDHB
     1  MAAVSGLVRR PLREVSGLLK RRFHWTAPAA LQVTVRDAIN QGMDEELERD EKVFLLGEEV
    61  AQYDGAYKVS RGLWKKYGDK RIIDTPISEM GFAGIAVGAA MAGLRPICEF MTFNFSMQAI
   121  DQVINSAAKT YYMSGGLQPV PIVFRGPNGA SAGVAAQHSQ CFAAWYGHCP GLKVVSPWNS
   181  EDAKGLIKSA IRDNNPVVVL ENELMYGVPF EFPPEAQSKD FLIPIGKAKI ERQGTHITVV
   241  SHSRPVGHCL EAAAVLSKEG VECEVINMRT IRPMDMETIE ASVMKTNHLV TVEGGWPQFG
   301  VGAEICARIM EGPAFNFLDA PAVRVTGADV PMPYAKILED NSIPQVKDII FAIKKTLNI

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against PDHB 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)
0.26
Highest tissue expression
279 nTPM

Expression across tissuesHPA

Tissue

  • tongue: 279 nTPM
  • skeletal muscle: 255 nTPM
  • heart muscle: 164 nTPM
  • choroid plexus: 125 nTPM
  • liver: 104 nTPM
  • kidney: 94 nTPM

Single-cell type

  • cytotrophoblasts: 205 nCPM
  • syncytiotrophoblasts: 176 nCPM
  • esophageal suprabasal cells: 167 nCPM
  • migrating cytotrophoblasts: 166 nCPM
  • parietal cells: 164 nCPM
  • esophageal apical cells: 153 nCPM

Immune cell

  • myeloid DC: 141 nTPM
  • NK-cell: 130 nTPM
  • total PBMC: 124 nTPM
  • intermediate monocyte: 115 nTPM
  • T-reg: 114 nTPM
  • non-classical monocyte: 110 nTPM

Brain region

  • choroid plexus: 62 nTPM
  • white matter: 41 nTPM
  • cerebellum: 40 nTPM
  • thalamus: 38 nTPM
  • hypothalamus: 37 nTPM
  • cerebral cortex: 37 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about PDHB.

Disease | AllUniProt

Conditions PDHB is implicated in, by any mechanism.

  • Pyruvate dehydrogenase E1-beta deficiency (PDHBD) MIM:614111

Disease | GeneticClinVar

44 pathogenic / likely-pathogenic of 444 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

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)
0.55
gnomAD pLI
0.15
gnomAD missense Z
1.57
DepMap mean gene effect
-0.11
DepMap dependency class
selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% 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 PDHB 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 PDHB as an antibody target. Whether an autoantibody or antibody against PDHB could matter depends on whether native PDHB is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

PDHB 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 PDHB as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/PDHB. 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...