MT-ND5

Protein identityUniProt · HPA

UniProt accession

P03915

Gene

MT-ND5

Ensembl

ENSG00000198786

Chromosome

MT

Canonical length

603 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; Leigh disease; and MELAS syndrome. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

603 residues, UniProt reviewed canonical sequence.

>P03915|MT-ND5
     1  MTMHTTMTTL TLTSLIPPIL TTLVNPNKKN SYPHYVKSIV ASTFIISLFP TTMFMCLDQE
    61  VIISNWHWAT TQTTQLSLSF KLDYFSMMFI PVALFVTWSI MEFSLWYMNS DPNINQFFKY
   121  LLIFLITMLI LVTANNLFQL FIGWEGVGIM SFLLISWWYA RADANTAAIQ AILYNRIGDI
   181  GFILALAWFI LHSNSWDPQQ MALLNANPSL TPLLGLLLAA AGKSAQLGLH PWLPSAMEGP
   241  TPVSALLHSS TMVVAGIFLL IRFHPLAENS PLIQTLTLCL GAITTLFAAV CALTQNDIKK
   301  IVAFSTSSQL GLMMVTIGIN QPHLAFLHIC THAFFKAMLF MCSGSIIHNL NNEQDIRKMG
   361  GLLKTMPLTS TSLTIGSLAL AGMPFLTGFY SKDHIIETAN MSYTNAWALS ITLIATSLTS
   421  AYSTRMILLT LTGQPRFPTL TNINENNPTL LNPIKRLAAG SLFAGFLITN NISPASPFQT
   481  TIPLYLKLTA LAVTFLGLLT ALDLNYLTNK LKMKSPLCTF YFSNMLGFYP SITHRTIPYL
   541  GLLTSQNLPL LLLDLTWLEK LLPKTISQHQ ISTSIITSTQ KGMIKLYFLS FFFPLILTLL
   601  LIT

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-ND5 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

15

Mean surface accessibility (rSASA)

0.24

Highest tissue expression

26,165 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-ND5.

Disease | AllUniProt

Conditions MT-ND5 is implicated in, by any mechanism.

• Leber hereditary optic neuropathy (LHON) MIM:535000
• Leigh syndrome (LS) MIM:256000
• Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) MIM:540000

Disease | GeneticClinVar

11 pathogenic / likely-pathogenic of 331 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• MELAS syndrome
• Mitochondrial disease
• Leber optic atrophy
• Primary Mitochondrial Disorders
• Leigh syndrome

OntologyGO

Biological processes

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

Molecular functions

• NADH dehydrogenase (ubiquinone) activity

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 (complex I), chain 5 N-terminal
• NADH-quinone oxidoreductase, chain 5-like
• NADH dehydrogenase subunit 5, C-terminal
• NADH-plastoquinone oxidoreductase, chain 5 subgroup
• NADH-Ubiquinone oxidoreductase (complex I), chain 5 N-terminus
• NADH dehydrogenase subunit 5 C-terminus

KeywordsUniProt

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