Seroatlas · Human Serome Atlas

MT-ND4

NADH-ubiquinone oxidoreductase chain 4

Also known as: LHON, MTND4, NAD4, ND4, NU4M_HUMAN

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

Protein identityUniProt · HPA

UniProt accession
P03905
Gene
MT-ND4
Ensembl
ENSG00000198886
Chromosome
MT
Canonical length
459 aa
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted membrane proteins
Subcellular location
Mitochondria

OverviewNCBI Gene

Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Located in mitochondrial inner membrane. Part of respiratory chain complex I. Implicated in Leber hereditary optic neuropathy; Parkinson's disease; macular degeneration; and schizophrenia. Biomarker of Alzheimer's disease. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

459 residues, UniProt reviewed canonical sequence.

>P03905|MT-ND4
     1  MLKLIVPTIM LLPLTWLSKK HMIWINTTTH SLIISIIPLL FFNQINNNLF SCSPTFSSDP
    61  LTTPLLMLTT WLLPLTIMAS QRHLSSEPLS RKKLYLSMLI SLQISLIMTF TATELIMFYI
   121  FFETTLIPTL AIITRWGNQP ERLNAGTYFL FYTLVGSLPL LIALIYTHNT LGSLNILLLT
   181  LTAQELSNSW ANNLMWLAYT MAFMVKMPLY GLHLWLPKAH VEAPIAGSMV LAAVLLKLGG
   241  YGMMRLTLIL NPLTKHMAYP FLVLSLWGMI MTSSICLRQT DLKSLIAYSS ISHMALVVTA
   301  ILIQTPWSFT GAVILMIAHG LTSSLLFCLA NSNYERTHSR IMILSQGLQT LLPLMAFWWL
   361  LASLANLALP PTINLLGELS VLVTTFSWSN ITLLLTGLNM LVTALYSLYM FTTTQWGSLT
   421  HHINNMKPSF TRENTLMFMH LSPILLLSLN PDIITGFSS

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-ND4 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
11
Mean surface accessibility (rSASA)
0.2
Highest tissue expression
197,809 nTPM

Expression across tissuesHPA

Tissue

  • heart muscle: 197,809 nTPM
  • liver: 99,476 nTPM
  • basal ganglia: 96,259 nTPM
  • hippocampal formation: 88,293 nTPM
  • amygdala: 79,077 nTPM
  • kidney: 74,839 nTPM

Single-cell type

  • hepatocytes: 36,704 nCPM
  • colonocytes: 31,375 nCPM
  • enterocytes: 26,410 nCPM
  • retinal bipolar cells: 25,444 nCPM
  • paneth cells: 24,496 nCPM
  • goblet cells: 23,341 nCPM

Immune cell

  • basophil: 13,564 nTPM
  • eosinophil: 7,119 nTPM
  • naive B-cell: 3,637 nTPM
  • memory B-cell: 3,568 nTPM
  • classical monocyte: 3,337 nTPM
  • naive CD8 T-cell: 3,105 nTPM

Brain region

  • medulla oblongata: 85,010 nTPM
  • choroid plexus: 84,793 nTPM
  • cerebral cortex: 79,859 nTPM
  • pons: 77,413 nTPM
  • thalamus: 66,587 nTPM
  • midbrain: 64,388 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-ND4.

Disease | AllUniProt

Conditions MT-ND4 is implicated in, by any mechanism.

  • Leber hereditary optic neuropathy (LHON) MIM:535000
  • Leber hereditary optic neuropathy with dystonia (LDYT) MIM:500001
  • Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) MIM:540000

Disease | GeneticClinVar

2 pathogenic / likely-pathogenic of 150 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads MT-ND4 as an antibody target. Whether an autoantibody or antibody against MT-ND4 could matter depends on whether native MT-ND4 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

MT-ND4 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 MT-ND4 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/MT-ND4. 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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