NDUFB8

Protein identityUniProt · HPA

UniProt accession

O95169

Gene

NDUFB8

Ensembl

ENSG00000166136

Chromosome

10

Canonical length

186 aa

Protein class

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

Subcellular location

Mitochondria

OverviewNCBI Gene

Predicted to enable NADH dehydrogenase (ubiquinone) activity. Predicted to be involved in mitochondrial electron transport, NADH to ubiquinone and proton motive force-driven mitochondrial ATP synthesis. Located in mitochondrial inner membrane. Part of respiratory chain complex I. Implicated in nuclear type mitochondrial complex I deficiency 32. Biomarker of Alzheimer's disease and Parkinson's disease. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

186 residues, UniProt reviewed canonical sequence.

>O95169|NDUFB8
     1  MAVARAGVLG VQWLQRASRN VMPLGARTAS HMTKDMFPGP YPRTPEERAA AAKKYNMRVE
    61  DYEPYPDDGM GYGDYPKLPD RSQHERDPWY SWDQPGLRLN WGEPMHWHLD MYNRNRVDTS
   121  PTPVSWHVMC MQLFGFLAFM IFMCWVGDVY PVYQPVGPKQ YPYNNLYLER GGDPSKEPER
   181  VVHYEI

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against NDUFB8 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)

0.48

Highest tissue expression

563 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about NDUFB8.

Disease | AllUniProt

Conditions NDUFB8 is implicated in, by any mechanism.

• Mitochondrial complex I deficiency, nuclear type 32 (MC1DN32) MIM:618252

Disease | GeneticClinVar

4 pathogenic / likely-pathogenic of 107 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial complex I deficiency, nuclear type 32

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)

0.98

gnomAD pLI

0.01

gnomAD missense Z

-0.11

DepMap mean gene effect

-0.37

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% 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
• proton motive force-driven mitochondrial ATP synthesis

Molecular functions

• NADH dehydrogenase (ubiquinone) activity

Cellular components

• mitochondrial inner membrane
• mitochondrial matrix
• mitochondrion
• respiratory chain complex I

Protein domainsUniProt · Pfam · InterPro

• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, metazoa
• NADH-ubiquinone oxidoreductase ASHI subunit (CI-ASHI or NDUFB8)

KeywordsUniProt

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

InteractionsUniProt · HPA

Protein binding partners of NDUFB8 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• DNAJC30
• NDUFA9
• NDUFB5