Seroatlas · Human Serome Atlas

NDUFB3

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3

Also known as: B12, NDUB3_HUMAN

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

Protein identityUniProt · HPA

UniProt accession
O43676
Gene
NDUFB3
Ensembl
ENSG00000119013
Chromosome
2
Canonical length
98 aa
Protein class
Disease related genes, Human disease related genes, Metabolic proteins, Predicted membrane proteins

OverviewNCBI Gene

This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which is the first enzyme in the electron transport chain of mitochondria. This protein localizes to the inner membrane of the mitochondrion as a single-pass membrane protein. Mutations in this gene contribute to mitochondrial complex 1 deficiency. Alternative splicing results in multiple transcript variants encoding the same protein. Humans have multiple pseudogenes of this gene. [provided by RefSeq, Mar 2012]

Canonical amino-acid sequenceUniProt

98 residues, UniProt reviewed canonical sequence.

>O43676|NDUFB3
     1  MAHEHGHEHG HHKMELPDYR QWKIEGTPLE TIQKKLAAKG LRDPWGRNEA WRYMGGFAKS
    61  VSFSDVFFKG FKWGFAAFVV AVGAEYYLES LNKDKKHH

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against NDUFB3 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
Other membrane
Secreted
No
Transmembrane segments
1
Mean surface accessibility (rSASA)
0.53
Highest tissue expression
677 nTPM

Expression across tissuesHPA

Tissue

  • tongue: 677 nTPM
  • skeletal muscle: 659 nTPM
  • heart muscle: 491 nTPM
  • kidney: 251 nTPM
  • parathyroid gland: 204 nTPM
  • choroid plexus: 197 nTPM

Single-cell type

  • esophageal apical cells: 768 nCPM
  • parietal cells: 739 nCPM
  • esophageal suprabasal cells: 596 nCPM
  • late primary spermatocytes: 487 nCPM
  • colonocytes: 459 nCPM
  • enterocytes: 441 nCPM

Immune cell

  • neutrophil: 265 nTPM
  • non-classical monocyte: 214 nTPM
  • plasmacytoid DC: 212 nTPM
  • intermediate monocyte: 205 nTPM
  • basophil: 204 nTPM
  • T-reg: 183 nTPM

Brain region

  • cerebellum: 99 nTPM
  • hypothalamus: 98 nTPM
  • choroid plexus: 91 nTPM
  • white matter: 86 nTPM
  • medulla oblongata: 84 nTPM
  • pons: 83 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about NDUFB3.

Disease | AllUniProt

Conditions NDUFB3 is implicated in, by any mechanism.

  • Mitochondrial complex I deficiency, nuclear type 25 (MC1DN25) MIM:618246

Disease | GeneticClinVar

5 pathogenic / likely-pathogenic of 49 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)
1.94
gnomAD pLI
0
gnomAD missense Z
0.32
DepMap mean gene effect
-0.65
DepMap dependency class
common

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% 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

  • NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
  • NADH-ubiquinone oxidoreductase B12 subunit family

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of NDUFB3 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 NDUFB3 as an antibody target. Whether an autoantibody or antibody against NDUFB3 could matter depends on whether native NDUFB3 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

NDUFB3 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 NDUFB3 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/NDUFB3. 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).

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