MT-ND3
NADH-ubiquinone oxidoreductase chain 3
Also known as: MTND3, NAD3, ND3, NU3M_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- P03897
- Gene
- MT-ND3
- Ensembl
- ENSG00000198840
- Chromosome
- MT
- Canonical length
- 115 aa
- Protein class
- Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted membrane proteins
- Subcellular location
- Cytosol
OverviewNCBI Gene
Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone. Located in mitochondrial inner membrane. Part of respiratory chain complex I. Implicated in Leber hereditary optic neuropathy; Leigh disease; and Parkinson's disease. [provided by Alliance of Genome Resources, Jul 2025]
Canonical amino-acid sequenceUniProt
115 residues, UniProt reviewed canonical sequence.
>P03897|MT-ND3
1 MNFALILMIN TLLALLLMII TFWLPQLNGY MEKSTPYECG FDPMSPARVP FSMKFFLVAI
61 TFLLFDLEIA LLLPLPWALQ TTNLPLMVMS SLLLIIILAL SLAYEWLQKG LDWTELocalizationUniProt · AlphaFold · HPA
Whether an antibody against MT-ND3 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
- 3
- Mean surface accessibility (rSASA)
- 0.49
- Highest tissue expression
- 186,830 nTPM
Expression across tissuesHPA
Tissue
- heart muscle: 186,830 nTPM
- basal ganglia: 89,168 nTPM
- kidney: 83,803 nTPM
- hippocampal formation: 73,129 nTPM
- liver: 70,972 nTPM
- amygdala: 70,176 nTPM
Single-cell type
- pancreatic acinar cells: 31,598 nCPM
- hepatocytes: 22,176 nCPM
- enterocytes: 21,790 nCPM
- urothelial cells: 16,494 nCPM
- endometrial glandular cells: 16,457 nCPM
- paneth cells: 16,375 nCPM
Immune cell
- basophil: 2,290 nTPM
- neutrophil: 978 nTPM
- naive CD8 T-cell: 772 nTPM
- naive B-cell: 689 nTPM
- memory B-cell: 675 nTPM
- classical monocyte: 617 nTPM
Brain region
- cerebral cortex: 52,866 nTPM
- medulla oblongata: 50,530 nTPM
- pons: 45,569 nTPM
- hypothalamus: 43,577 nTPM
- thalamus: 43,208 nTPM
- midbrain: 41,365 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about MT-ND3.
Disease | AllUniProt
Conditions MT-ND3 is implicated in, by any mechanism.
- Leigh syndrome (LS) MIM:256000
- Mitochondrial complex I deficiency, mitochondrial type 1 (MC1DM1) MIM:500014
Disease | GeneticClinVar
5 pathogenic / likely-pathogenic of 52 ClinVar records.
Conditions with pathogenic or likely-pathogenic variants.
- Leigh syndrome
- Primary Mitochondrial Disorders
- Mitochondrial disease
- Mitochondrial complex I deficiency, mitochondrial type 1
- Mitochondrial complex I deficiency
OntologyGO
Biological processes
- aerobic respiration
- cellular response to glucocorticoid stimulus
- mitochondrial electron transport, NADH to ubiquinone
- proton motive force-driven mitochondrial ATP synthesis
- response to light intensity
- response to oxidative stress
Molecular functions
Cellular components
Protein domainsUniProt · Pfam · InterPro
- NADH:ubiquinone/plastoquinone oxidoreductase, chain 3
- NADH:ubiquinone oxidoreductase, subunit 3 superfamily
- NADH-ubiquinone/plastoquinone oxidoreductase, chain 3
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of MT-ND3 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 MT-ND3 as an antibody target. Whether an autoantibody or antibody against MT-ND3 could matter depends on whether native MT-ND3 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
MT-ND3 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-ND3 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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