Gene
NDUFA11
NADH:ubiquinone oxidoreductase subunit A11
- HGNC 20371↗(opens in a new tab)
- Ensembl gene ENSG00000174886↗(opens in a new tab)
- NCBI Gene 126328↗(opens in a new tab)
Summary
- Cytogenetic location
- 19p13.3
- Coordinates
- Chromosome 19: 5,894,675–5,903,790 (GRCh38)
- Exons
- 4 exons across 9.1 kb
- Protein
- 141 amino acids
- Also known as
- B14.7
- MANE Select transcript
- ENST00000308961.5NM_175614.5
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Primary Mitochondrial Disease
- Mitochondrial Respiratory Chain Complex Deficiency
One of 61 genes linked to it
Isolated deficiencies of complex I, II, III, IV or V, caused by variants in nuclear or mitochondrial genes, which can affect the brain, muscles, heart, liver and other energy-demanding organs.
- 1 GenCC submitter rates it definitive or strong
What knowing changes
Knowing can end a diagnostic search and guide surveillance of affected organs and avoidance of drugs toxic to mitochondria.
How NDUFA11 is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Stretches short reads find hard
Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.
Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).
Nearby genes from Ensembl release 116, on GRCh38.
Exons make up 0.6 kb of the gene's 9.1 kb; 426 of those bases code for the protein.
How a genome test reads NDUFA11
1
position in NDUFA11 where ClinVar classes a change as pathogenic or likely pathogenic
None of them falls in a region short reads find hard.
A whole genome covers all 9.1 kb of NDUFA11, introns included, not only the 0.6 kb of exons.
Genes linked to the same conditions
- ACAD9acyl-CoA dehydrogenase family member 9Shares 1 condition
- ATP5F1AATP synthase F1 subunit alphaShares 1 condition
- ATP5F1DATP synthase F1 subunit deltaShares 1 condition
- ATP5F1EATP synthase F1 subunit epsilonShares 1 condition
- BCS1LBCS1 ubiquinol-cytochrome c reductase complex chaperoneShares 1 condition
- COA6cytochrome c oxidase assembly factor 6Shares 1 condition
- COA8cytochrome c oxidase assembly factor 8Shares 1 condition
- COX10cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10Shares 1 condition
- COX15cytochrome c oxidase assembly factor COX15Shares 1 condition
- COX16cytochrome c oxidase assembly factor COX16Shares 1 condition
- COX20cytochrome c oxidase assembly factor COX20Shares 1 condition
- COX6B1cytochrome c oxidase subunit 6B1Shares 1 condition
Traits linked to NDUFA11
The Human Phenotype Ontology lists these as features of the rare conditions NDUFA11 can cause. Each trait's page lists the other genes linked to it.
Abnormality of the nervous system
- Brain degeneration
- Global developmental delay
- Ataxia
- Encephalopathy
- Focal T2 hyperintense brainstem lesion
- Lethargy
- Leukodystrophy
- Leukoencephalopathy
Show 2 more traits
External links
Gene records
Transcript
Protein
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). NDUFA11 gene: NADH:ubiquinone oxidoreductase subunit A11. Retrieved 17 October 2026, from https://dantelabs.com/genes/ndufa11
Dante Labs. NDUFA11 gene: NADH:ubiquinone oxidoreductase subunit A11 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/ndufa11
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