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Gene

NDUFB11

NADH:ubiquinone oxidoreductase subunit B11

Summary

Cytogenetic location
Xp11.3
Coordinates
Chromosome X: 47,142,216–47,144,702 (GRCh38)
Exons
3 exons across 2.5 kb
Protein
153 amino acids
Also known as
ESSS, NP17.3, Np15
MANE Select transcript
ENST00000377811.4NM_001135998.3
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Microphthalmia, Anophthalmia & Coloboma

    • Syndromic Microphthalmia

      Microphthalmia occurring with features outside the eye, caused by changes in genes such as SOX2, OTX2, BCOR, STRA6 and BMP4.

      • 1 GenCC submitter rates it definitive or strong
      What knowing changes

      A genetic diagnosis guides checks for associated features, such as pituitary hormone problems with SOX2 or OTX2, and informs recurrence risk.

Broader groupings

  • 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.

      • 2 GenCC submitters rate 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 NDUFB11 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
Chromosome X, with a bracket where NDUFB11 sits. NDUFB11 and 15 genes near it on chromosome X, from 46,643,459 to 47,643,458.Chromosome XXp11.3p22.33p22.32p22.31p22.2p22.13p22.12p22.11p21.3p21.2p21.1p11.4p11.3p11.23p11.22p11.21p11.1q11.1q11.2q12q13.1q13.2q13.3q21.1q21.2q21.31q21.32q21.33q22.1q22.2q22.3q23q24q25q26.1q26.2q26.3q27.1q27.2q27.3q2846.8 Mb47 Mb47.2 Mb47.4 Mb47.6 MbSLC9A7: 46,599,251–46,759,215, reverse strandSLC9A7RP2: 46,836,922–46,889,240, forward strandRP2JADE3: 46,912,276–47,061,242, forward strandJADE3RGN: 47,061,388–47,096,431, forward strandRGNNDUFB11: 47,142,216–47,144,702, reverse strandNDUFB11RBM10: 47,144,284–47,186,814, forward strandRBM10UBA1: 47,190,713–47,215,173, forward strandUBA1CDK16: 47,217,860–47,230,000, forward strandCDK16USP11: 47,232,866–47,248,328, forward strandUSP11ZNF157: 47,366,479–47,414,624, forward strandZNF157ZNF41: 47,444,693–47,483,247, reverse strandZNF41ARAF: 47,561,043–47,571,921, forward strandARAFSYN1: 47,571,890–47,619,970, reverse strandSYN1TIMP1: 47,582,227–47,586,793, forward strandTIMP1CFP: 47,621,537–47,630,305, reverse strandCFPELK1: 47,631,617–47,650,604, reverse strandELK1Diagram of NDUFB11: 3 exons on the reverse strand of chromosome XExon 3: 2 known disease-causing sitesExon 2: 3 known disease-causing sitesExon 1: 1 known disease-causing siteExon 3: 47,142,216–47,142,440, 225 basesExon 2: 47,142,614–47,142,744, 131 basesExon 1: 47,144,473–47,144,702, 230 bases3215′3′

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 2.5 kb; 462 of those bases code for the protein.

How a genome test reads NDUFB11

8

positions in NDUFB11 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 2.5 kb of NDUFB11, introns included, not only the 0.6 kb of exons.

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

APA

Dante Labs. (n.d.). NDUFB11 gene: NADH:ubiquinone oxidoreductase subunit B11. Retrieved 17 October 2026, from https://dantelabs.com/genes/ndufb11

Vancouver

Dante Labs. NDUFB11 gene: NADH:ubiquinone oxidoreductase subunit B11 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/ndufb11

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