MT-ND1

Protein identityUniProt · HPA

UniProt accession

P03886

Gene

MT-ND1

Ensembl

ENSG00000198888

Chromosome

MT

Canonical length

318 aa

Protein class

Disease related genes, Enzymes, FDA approved drug targets, Human disease related genes, Metabolic proteins, 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 several diseases, including MELAS syndrome; neurodegenerative disease (multiple); optic nerve disease (multiple); toxic shock syndrome; and type 2 diabetes mellitus. Biomarker of Alzheimer's disease; Parkinson's disease; and multiple sclerosis. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

318 residues, UniProt reviewed canonical sequence.

>P03886|MT-ND1
     1  MPMANLLLLI VPILIAMAFL MLTERKILGY MQLRKGPNVV GPYGLLQPFA DAMKLFTKEP
    61  LKPATSTITL YITAPTLALT IALLLWTPLP MPNPLVNLNL GLLFILATSS LAVYSILWSG
   121  WASNSNYALI GALRAVAQTI SYEVTLAIIL LSTLLMSGSF NLSTLITTQE HLWLLLPSWP
   181  LAMMWFISTL AETNRTPFDL AEGESELVSG FNIEYAAGPF ALFFMAEYTN IIMMNTLTTT
   241  IFLGTTYDAL SPELYTTYFV TKTLLLTSLF LWIRTAYPRF RYDQLMHLLW KNFLPLTLAL
   301  LMWYVSMPIT ISSIPPQT

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-ND1 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

8

Mean surface accessibility (rSASA)

0.26

Highest tissue expression

129,323 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-ND1.

Disease | AllUniProt

Conditions MT-ND1 is implicated in, by any mechanism.

• Leber hereditary optic neuropathy (LHON) MIM:535000
• Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) MIM:540000
• Alzheimer disease mitochondrial (AD-MT) MIM:502500
• Type 2 diabetes mellitus (T2D) MIM:125853

Disease | GeneticClinVar

11 pathogenic / likely-pathogenic of 189 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial disease
• Primary Mitochondrial Disorders
• Leber optic atrophy
• Leber optic atrophy and dystonia
• Leigh syndrome

OntologyGO

Biological processes

• aerobic respiration
• mitochondrial electron transport, NADH to ubiquinone
• mitochondrial respiratory chain complex I assembly
• proton motive force-driven mitochondrial ATP synthesis
• response to hydroperoxide
• response to hypoxia
• response to xenobiotic stimulus

Molecular functions

• NADH dehydrogenase (ubiquinone) activity

Cellular components

• dendrite
• mitochondrial inner membrane
• mitochondrial membrane
• mitochondrion
• neuronal cell body
• respiratory chain complex I

Protein domainsUniProt · Pfam · InterPro

• NADH:ubiquinone oxidoreductase, subunit 1/F420H2 oxidoreductase subunit H
• NADH:ubiquinone oxidoreductase, subunit 1, conserved site
• NADH dehydrogenase

KeywordsUniProt

• Alzheimer disease
• Amyloidosis
• Diabetes mellitus
• Electron transport
• Epilepsy
• Leber hereditary optic neuropathy
• MELAS 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-ND1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• NAMPT