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Gene

UQCRB

ubiquinol-cytochrome c reductase binding protein

Summary

Cytogenetic location
8q22.1
Coordinates
Chromosome 8: 96,222,947–96,235,545 (GRCh38)
Exons
4 exons across 12.6 kb
Protein
111 amino acids
Also known as
QP-C, QCR7, UQCR6
Previous symbols
UQBP
MANE Select transcript
ENST00000287022.10NM_006294.5
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 report relevant to this gene

    • 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 UQCRB is laid out

    • Coding sequence
    • Untranslated region
    • Intron
    • Direction of transcription
    • Known disease-causing sites
    • Stretches short reads find hard
    Chromosome 8, with a bracket where UQCRB sits. UQCRB and 5 genes near it on chromosome 8, from 95,729,246 to 96,729,245.Chromosome 88q22.1p23.3p23.2p23.1p22p21.3p21.2p21.1p12p11.23p11.22p11.21p11.1q11.1q11.21q11.22q11.23q12.1q12.2q12.3q13.1q13.2q13.3q21.11q21.12q21.13q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.3q24.11q24.12q24.13q24.21q24.22q24.23q24.395.8 Mb96 Mb96.2 Mb96.4 Mb96.6 MbGDF6: 96,142,333–96,160,813, reverse strandGDF6UQCRB: 96,222,947–96,235,545, reverse strandUQCRBMTERF3: 96,238,162–96,261,626, reverse strandMTERF3PTDSS1: 96,261,885–96,386,078, forward strandPTDSS1SDC2: 96,493,410–96,613,465, forward strandSDC2CPQ: 96,644,982–97,152,036, forward strandCPQDiagram of UQCRB: 4 exons on the reverse strand of chromosome 8Exon 4: 1 known disease-causing siteExon 4: 96,222,947–96,231,132, 8,186 basesExon 3: 96,231,774–96,231,940, 167 basesExon 2: 96,233,156–96,233,227, 72 basesExon 1: 96,235,512–96,235,545, 34 bases43215′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 8.5 kb of the gene's 12.6 kb; 336 of those bases code for the protein.

    How a genome test reads UQCRB

    1

    position in UQCRB where ClinVar classes a change as pathogenic or likely pathogenic

    1 of them (100%) falls in a region short reads find hard.

    • Segmental duplications (1 site)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.

    A whole genome covers all 12.6 kb of UQCRB, introns included, not only the 8.5 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.). UQCRB gene: ubiquinol-cytochrome c reductase binding protein. Retrieved 17 October 2026, from https://dantelabs.com/genes/uqcrb

    Vancouver

    Dante Labs. UQCRB gene: ubiquinol-cytochrome c reductase binding protein [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/uqcrb

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