MT-ND2

Protein identityUniProt · HPA

UniProt accession

P03891

Gene

MT-ND2

Ensembl

ENSG00000198763

Chromosome

MT

Canonical length

347 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted membrane proteins

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; multiple sclerosis; myocardial infarction; neurodegenerative disease (multiple); and urinary bladder cancer. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

347 residues, UniProt reviewed canonical sequence.

>P03891|MT-ND2
     1  MNPLAQPVIY STIFAGTLIT ALSSHWFFTW VGLEMNMLAF IPVLTKKMNP RSTEAAIKYF
    61  LTQATASMIL LMAILFNNML SGQWTMTNTT NQYSSLMIMM AMAMKLGMAP FHFWVPEVTQ
   121  GTPLTSGLLL LTWQKLAPIS IMYQISPSLN VSLLLTLSIL SIMAGSWGGL NQTQLRKILA
   181  YSSITHMGWM MAVLPYNPNM TILNLTIYII LTTTAFLLLN LNSSTTTLLL SRTWNKLTWL
   241  TPLIPSTLLS LGGLPPLTGF LPKWAIIEEF TKNNSLIIPT IMATITLLNL YFYLRLIYST
   301  SITLLPMSNN VKMKWQFEHT KPTPFLPTLI ALTTLLLPIS PFMLMIL

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-ND2 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

10

Mean surface accessibility (rSASA)

0.23

Highest tissue expression

130,853 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-ND2.

Disease | AllUniProt

Conditions MT-ND2 is implicated in, by any mechanism.

• Leber hereditary optic neuropathy (LHON) MIM:535000
• Alzheimer disease mitochondrial (AD-MT) MIM:502500
• Leigh syndrome (LS) MIM:256000

Disease | GeneticClinVar

1 pathogenic / likely-pathogenic of 186 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial complex I deficiency
• Mitochondrial disease

OntologyGO

Biological processes

• aerobic respiration
• mitochondrial electron transport, NADH to ubiquinone
• mitochondrial respiratory chain complex I assembly
• proton motive force-driven mitochondrial ATP synthesis
• reactive oxygen species metabolic process
• response to hypoxia

Molecular functions

• ionotropic glutamate receptor binding
• NADH dehydrogenase (ubiquinone) activity
• protein kinase binding

Cellular components

• mitochondrial inner membrane
• mitochondrion
• respiratory chain complex I

Protein domainsUniProt · Pfam · InterPro

• NADH:quinone oxidoreductase/Mrp antiporter, transmembrane domain
• NADH:quinone oxidoreductase/Mrp antiporter, TM
• NADH:ubiquinone oxidoreductase, chain 2
• NADH dehydrogenase subunit 2, C-terminal
• Complex I Subunit 2
• NADH dehydrogenase subunit 2 C-terminus

KeywordsUniProt

• Alzheimer disease
• Amyloidosis
• Electron transport
• Leber hereditary optic neuropathy
• Leigh syndrome
• Membrane
• Mitochondrion
• Mitochondrion inner membrane
• NAD
• Neurodegeneration
• Primary mitochondrial disease
• Respiratory chain
• Translocase
• Transmembrane
• Transmembrane helix
• Transport
• Ubiquinone

InteractionsUniProt · HPA

Protein binding partners of MT-ND2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• TMEM242