NDUFC2

Protein identityUniProt · HPA

UniProt accession

O95298

Gene

NDUFC2

Ensembl

ENSG00000151366

Chromosome

11

Canonical length

119 aa

Protein class

Disease related genes, Human disease related genes, Metabolic proteins, Plasma proteins, Predicted intracellular proteins, Predicted membrane proteins

Subcellular location

Mitochondria

OverviewNCBI Gene

Predicted to enable NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial respiratory chain complex I assembly. Located in mitochondrial inner membrane. Part of respiratory chain complex I. Implicated in nuclear type mitochondrial complex I deficiency. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

119 residues, UniProt reviewed canonical sequence.

>O95298|NDUFC2
     1  MIARRNPEPL RFLPDEARSL PPPKLTDPRL LYIGFLGYCS GLIDNLIRRR PIATAGLHRQ
    61  LLYITAFFFA GYYLVKREDY LYAVRDREMF GYMKLHPEDF PEEDKKTYGE IFEKFHPIR

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against NDUFC2 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

1

Mean surface accessibility (rSASA)

0.43

Highest tissue expression

457 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about NDUFC2.

Disease | AllUniProt

Conditions NDUFC2 is implicated in, by any mechanism.

• Mitochondrial complex I deficiency, nuclear type 36 (MC1DN36) MIM:619170

Disease | GeneticClinVar

3 pathogenic / likely-pathogenic of 8 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial complex I deficiency, nuclear type 36
• Mitochondrial disease

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.8

gnomAD pLI

0.68

gnomAD missense Z

0.24

DepMap mean gene effect

-0.48

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 7% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• aerobic respiration
• mitochondrial electron transport, NADH to ubiquinone
• mitochondrial respiratory chain complex I assembly
• negative regulation of non-canonical NF-kappaB signal transduction
• negative regulation of reactive oxygen species biosynthetic process
• positive regulation of ATP biosynthetic process
• positive regulation of mitochondrial membrane potential
• proton motive force-driven mitochondrial ATP synthesis

Molecular functions

• NADH dehydrogenase (ubiquinone) activity

Cellular components

• azurophil granule membrane
• cytoplasm
• mitochondrial inner membrane
• mitochondrion
• plasma membrane
• respiratory chain complex I

Protein domainsUniProt · Pfam · InterPro

• NADH dehydrogenase [ubiquinone] 1 subunit C2, NDUC2
• NADH-ubiquinone oxidoreductase subunit b14.5b (NDUFC2)

KeywordsUniProt

• Electron transport
• Membrane
• Mitochondrion
• Mitochondrion inner membrane
• Primary mitochondrial disease
• Respiratory chain
• Transmembrane
• Transmembrane helix
• Transport

InteractionsUniProt · HPA

Protein binding partners of NDUFC2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• TMEM242
• NDUFA13
• NDUFA9
• NDUFB10
• NDUFB5
• NDUFS7