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Aliases for UQCRB Gene

Aliases for UQCRB Gene

  • Ubiquinol-Cytochrome C Reductase Binding Protein 2 3 5
  • Ubiquinol-Cytochrome C Reductase, Complex III Subunit VI 2 3
  • Ubiquinol-Cytochrome C Reductase Complex 14 KDa Protein 3 4
  • Cytochrome B-C1 Complex Subunit 7 2 3
  • Complex III Subunit VII 3 4
  • Complex III Subunit 7 3 4
  • UQBP 3 4
  • QP-C 3 4
  • Mitochondrial Ubiquinone-Binding Protein 3
  • MC3DN3 3
  • UQCR6 3
  • QCR7 3
  • UQBC 3
  • UQPC 3
  • QPC 3

External Ids for UQCRB Gene

Previous HGNC Symbols for UQCRB Gene

  • UQBP

Previous GeneCards Identifiers for UQCRB Gene

  • GC08M096251
  • GC08M097316
  • GC08M096911
  • GC08M097199
  • GC08M097311
  • GC08M092444

Summaries for UQCRB Gene

Entrez Gene Summary for UQCRB Gene

  • This gene encodes a subunit of the ubiquinol-cytochrome c oxidoreductase complex, which consists of one mitochondrial-encoded and 10 nuclear-encoded subunits. The protein encoded by this gene binds ubiquinone and participates in the transfer of electrons when ubiquinone is bound. This protein plays an important role in hypoxia-induced angiogenesis through mitochondrial reactive oxygen species-mediated signaling. Mutations in this gene are associated with mitochondrial complex III deficiency. Alternatively spliced transcript variants have been found for this gene. Related pseudogenes have been identified on chromosomes 1, 5 and X. [provided by RefSeq, Dec 2011]

GeneCards Summary for UQCRB Gene

UQCRB (Ubiquinol-Cytochrome C Reductase Binding Protein) is a Protein Coding gene. Diseases associated with UQCRB include Mitochondrial Complex Iii Deficiency, Nuclear Type 3 and Mitochondrial Complex Iii Deficiency. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Metabolism.

UniProtKB/Swiss-Prot for UQCRB Gene

  • This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This component is involved in redox-linked proton pumping.

Gene Wiki entry for UQCRB Gene

Additional gene information for UQCRB Gene

No data available for CIViC summary , Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for UQCRB Gene

Genomics for UQCRB Gene

GeneHancer (GH) Regulatory Elements for UQCRB Gene

Promoters and enhancers for UQCRB Gene
GeneHancer (GH) Identifier GH Type GH
GH Sources Gene Association Score Total Score TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor
Binding Sites
Gene Targets
GH08I096233 Promoter/Enhancer 2 EPDnew Ensembl ENCODE 550.8 +0.5 539 3.1 HDGF SMAD1 FOXA2 ARNT ARID4B SIN3A DMAP1 ZBTB7B YY1 POLR2B LOC101927039 UQCRB MTERF3 PTDSS1 GC08P096183
GH08I096267 Enhancer 0.9 Ensembl ENCODE 11.8 -33.3 -33257 3.4 MAX ZMYM3 ZNF384 RAD21 HNF4G ZSCAN29 FOS GATAD2A SOX6 RXRA GC08P096270 LOC101927039 UQCRB PTDSS1 LOC105375652
GH08I096231 Enhancer 0.6 ENCODE 0.8 +3.8 3846 0.2 SRF PKNOX1 NR2F1 JUNB DPF2 ATF2 IRF4 ZNF217 BCL11A IKZF1 MTERF3 PTDSS1 GDF6 LOC101927039 UQCRB GC08P096183
GH08I096227 Enhancer 0.7 ENCODE 0.4 +8.3 8306 0.2 CTCF ZNF654 TRIM22 CEBPB CEBPG REST RAD21 ATF2 HIC1 ZNF366 MTERF3 PTDSS1 GDF6 UQCRB LOC101927039 GC08P096183
GH08I096259 Promoter 0.7 EPDnew 0.3 -23.5 -23455 0.1 GATA3 IKZF1 REST MTERF3 UQCRB
- Elite GeneHancer and/or Elite GeneHancer-gene association Download GeneHancer data dump

GeneHancers around UQCRB on UCSC Golden Path with GeneCards custom track

Top Transcription factor binding sites by QIAGEN in the UQCRB gene promoter:

Genomic Locations for UQCRB Gene

Genomic Locations for UQCRB Gene
9,715 bases
Minus strand

Genomic View for UQCRB Gene

Genes around UQCRB on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
UQCRB Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for UQCRB Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for UQCRB Gene

Proteins for UQCRB Gene

  • Protein details for UQCRB Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Cytochrome b-c1 complex subunit 7
    Protein Accession:
    Secondary Accessions:
    • E5RJU0
    • Q6FGD1

    Protein attributes for UQCRB Gene

    111 amino acids
    Molecular mass:
    13530 Da
    Quaternary structure:
    • The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).

    Three dimensional structures from OCA and Proteopedia for UQCRB Gene

    Alternative splice isoforms for UQCRB Gene


neXtProt entry for UQCRB Gene

Post-translational modifications for UQCRB Gene

  • Ubiquitination at isoforms=212 and posLast=2929
  • Modification sites at PhosphoSitePlus

No data available for DME Specific Peptides for UQCRB Gene

Domains & Families for UQCRB Gene

Gene Families for UQCRB Gene

Protein Domains for UQCRB Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the UQCRB/QCR7 family.
  • Belongs to the UQCRB/QCR7 family.
genes like me logo Genes that share domains with UQCRB: view

Function for UQCRB Gene

Molecular function for UQCRB Gene

GENATLAS Biochemistry:
ubiquinol binding protein,13kDa,component of cytochrome bc1,complex III of mitochondrial respiratory chain,oxidative phosphorylation (OXPHOS)
UniProtKB/Swiss-Prot Function:
This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This component is involved in redox-linked proton pumping.

Gene Ontology (GO) - Molecular Function for UQCRB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005515 protein binding IPI 16189514
GO:0008121 contributes_to ubiquinol-cytochrome-c reductase activity IBA --
genes like me logo Genes that share ontologies with UQCRB: view
genes like me logo Genes that share phenotypes with UQCRB: view

Human Phenotype Ontology for UQCRB Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Models for UQCRB Gene

MGI Knock Outs for UQCRB:

Animal Model Products

CRISPR Products

miRNA for UQCRB Gene

miRTarBase miRNAs that target UQCRB

Inhibitory RNA Products

Clone Products

  • Addgene plasmids for UQCRB

No data available for Enzyme Numbers (IUBMB) , Phenotypes From GWAS Catalog , Transcription Factor Targets and HOMER Transcription for UQCRB Gene

Localization for UQCRB Gene

Subcellular locations from UniProtKB/Swiss-Prot for UQCRB Gene

Mitochondrion inner membrane.

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for UQCRB gene
Compartment Confidence
mitochondrion 5

Gene Ontology (GO) - Cellular Components for UQCRB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA --
GO:0005743 mitochondrial inner membrane TAS --
GO:0005746 mitochondrial respiratory chain TAS 3056408
GO:0005750 mitochondrial respiratory chain complex III IBA,IEA --
GO:0016020 membrane IEA --
genes like me logo Genes that share ontologies with UQCRB: view

No data available for Subcellular locations from the Human Protein Atlas (HPA) for UQCRB Gene

Pathways & Interactions for UQCRB Gene

genes like me logo Genes that share pathways with UQCRB: view

Gene Ontology (GO) - Biological Process for UQCRB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006119 oxidative phosphorylation TAS 3056408
GO:0006122 mitochondrial electron transport, ubiquinol to cytochrome c IEA,TAS --
GO:0009060 aerobic respiration TAS 3056408
GO:0034551 mitochondrial respiratory chain complex III assembly IBA --
GO:0055114 oxidation-reduction process TAS 3056408
genes like me logo Genes that share ontologies with UQCRB: view

No data available for SIGNOR curated interactions for UQCRB Gene

Drugs & Compounds for UQCRB Gene

(10) Drugs for UQCRB Gene - From: DrugBank and HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Ubiquinone-2 Experimental Pharma Target 0
(5S)-3-ANILINO-5-(2,4-DIFLUOROPHENYL)-5-METHYL-1,3-OXAZOLIDINE-2,4-DIONE Experimental Pharma Target 0
2-Hexyloxy-6-Hydroxymethyl-Tetrahydro-Pyran-3,4,5-Triol Experimental Pharma Target 0
2-NONYL-4-HYDROXYQUINOLINE N-OXIDE Experimental Pharma Target 0
5-HEPTYL-6-HYDROXY-1,3-BENZOTHIAZOLE-4,7-DIONE Experimental Pharma Target 0

(1) Additional Compounds for UQCRB Gene - From: HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
  • Coenzymes QH2
  • CoQH2
  • Reduced ubiquinone
  • Ubiquinol
  • Ubiquinone-1
genes like me logo Genes that share compounds with UQCRB: view

Transcripts for UQCRB Gene

Unigene Clusters for UQCRB Gene

Ubiquinol-cytochrome c reductase binding protein:
Representative Sequences:

CRISPR Products

Inhibitory RNA Products

Clone Products

  • Addgene plasmids for UQCRB

Alternative Splicing Database (ASD) splice patterns (SP) for UQCRB Gene

ExUns: 1a · 1b ^ 2 ^ 3a · 3b ^ 4 ^ 5a · 5b
SP1: - -
SP2: -
SP3: -

Relevant External Links for UQCRB Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for UQCRB Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and CGAP SAGE for UQCRB Gene

mRNA differential expression in normal tissues according to GTEx for UQCRB Gene

This gene is overexpressed in Heart - Left Ventricle (x4.5).

Protein differential expression in normal tissues from HIPED for UQCRB Gene

This gene is overexpressed in Brain (13.8) and Heart (7.1).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MaxQB, and MOPED for UQCRB Gene

Protein tissue co-expression partners for UQCRB Gene

- Elite partner

NURSA nuclear receptor signaling pathways regulating expression of UQCRB Gene:


SOURCE GeneReport for Unigene cluster for UQCRB Gene:


Evidence on tissue expression from TISSUES for UQCRB Gene

  • Liver(4.4)
  • Nervous system(4.4)
  • Muscle(3.7)
  • Bone(2.7)
  • Heart(2.6)
  • Adrenal gland(2.2)
  • Kidney(2.2)
  • Lymph node(2)
  • Skin(2)
  • Stomach(2)

Phenotype-based relationships between genes and organs from Gene ORGANizer for UQCRB Gene

Germ Layers:
  • ectoderm
  • endoderm
  • mesoderm
  • cardiovascular
  • digestive
  • endocrine
  • immune
  • integumentary
  • nervous
  • reproductive
  • respiratory
  • skeletal muscle
  • skeleton
  • urinary
Head and neck:
  • brain
  • cerebellum
  • cranial nerve
  • ear
  • eye
  • eyelid
  • face
  • forehead
  • head
  • larynx
  • mouth
  • neck
  • nose
  • skull
  • tongue
  • vocal cord
  • heart
  • heart valve
  • lung
  • abdominal wall
  • adrenal gland
  • biliary tract
  • gallbladder
  • kidney
  • liver
  • pancreas
  • testicle
  • arm
  • digit
  • finger
  • foot
  • hand
  • lower limb
  • toe
  • upper limb
  • blood
  • coagulation system
  • hair
  • peripheral nerve
  • peripheral nervous system
  • red blood cell
  • skin
  • spinal cord
  • sweat gland
  • white blood cell
genes like me logo Genes that share expression patterns with UQCRB: view

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA Expression by UniProt/SwissProt for UQCRB Gene

Orthologs for UQCRB Gene

This gene was present in the common ancestor of animals and fungi.

Orthologs for UQCRB Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia UQCRB 33 34
  • 99.1 (n)
(Bos Taurus)
Mammalia UQCRB 33 34
  • 89.19 (n)
(Mus musculus)
Mammalia Uqcrb 33 16 34
  • 88.29 (n)
(Canis familiaris)
Mammalia LOC477944 33
  • 87.88 (n)
  • 72 (a)
(Monodelphis domestica)
Mammalia UQCRB 34
  • 67 (a)
(Ornithorhynchus anatinus)
Mammalia UQCRB 34
  • 62 (a)
(Gallus gallus)
Aves UQCRB 33
  • 73.87 (n)
(Anolis carolinensis)
Reptilia UQCRB 34
  • 59 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia LOC101732809 33
  • 72.37 (n)
African clawed frog
(Xenopus laevis)
Amphibia Xl.24179 33
(Danio rerio)
Actinopterygii uqcrb 33 34
  • 68.77 (n)
Dr.3887 33
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.12392 33
fruit fly
(Drosophila melanogaster)
Insecta CG17856 33 34
  • 60.87 (n)
CG3560 34
  • 46 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP012188 33
  • 59.93 (n)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes QCR7 34 36
  • 28 (a)
Species where no ortholog for UQCRB was found in the sources mined by GeneCards:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rat (Rattus norvegicus)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)

Evolution for UQCRB Gene

Gene Tree for UQCRB (if available)
Gene Tree for UQCRB (if available)

Paralogs for UQCRB Gene Pseudogenes for UQCRB Gene

genes like me logo Genes that share paralogs with UQCRB: view

No data available for Paralogs for UQCRB Gene

Variants for UQCRB Gene

Sequence variations from dbSNP and Humsavar for UQCRB Gene

SNP ID Clin Chr 08 pos Variation AA Info Type
rs886043294 pathogenic, Mitochondrial complex III deficiency, nuclear type 3 96,231,082(-) TTTT/ 3_prime_UTR_variant, coding_sequence_variant, frameshift, non_coding_transcript_variant
rs863224259 pathogenic, not provided 96,231,117(-) C/ coding_sequence_variant, frameshift, non_coding_transcript_variant
rs863224257 likely-pathogenic, not provided 96,233,191(-) T/A 5_prime_UTR_variant, coding_sequence_variant, missense_variant, non_coding_transcript_variant
rs863224258 likely-pathogenic, not provided 96,231,067(-) C/A/T 3_prime_UTR_variant, coding_sequence_variant, missense_variant, non_coding_transcript_variant, stop_gained
rs1057523920 likely-benign, not specified 96,233,145(-) C/G intron_variant

Structural Variations from Database of Genomic Variants (DGV) for UQCRB Gene

Variant ID Type Subtype PubMed ID
nsv1020494 CNV gain 25217958
nsv6318 CNV insertion 18451855

Variation tolerance for UQCRB Gene

Residual Variation Intolerance Score: 65.9% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 3.35; 53.74% of all genes are more intolerant (likely to be disease-causing)

Additional Variant Information for UQCRB Gene

Human Gene Mutation Database (HGMD)
SNPedia medical, phenotypic, and genealogical associations of SNPs for

SNP Genotyping and Copy Number Assay Products

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for UQCRB Gene

Disorders for UQCRB Gene

MalaCards: The human disease database

(4) MalaCards diseases for UQCRB Gene - From: HGMD, OMIM, ClinVar, GTR, Orphanet, and GeneCards

Disorder Aliases PubMed IDs
mitochondrial complex iii deficiency, nuclear type 3
  • mc3dn3
mitochondrial complex iii deficiency
  • complex 3 mitochondrial respiratory chain deficiency
mitochondrial complex iii deficiency, nuclear type 1
  • mc3dn1
isolated complex iii deficiency
  • isolated coq-cytochrome c reductase deficiency
- elite association - COSMIC cancer census association via MalaCards
Search UQCRB in MalaCards View complete list of genes associated with diseases


  • Mitochondrial complex III deficiency, nuclear 3 (MC3DN3) [MIM:615158]: A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. {ECO:0000269 PubMed:12709789}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Additional Disease Information for UQCRB

Genetic Association Database
Human Genome Epidemiology Navigator
ATLAS of Genetics and Cytogenetics in Oncology and Haematology
genes like me logo Genes that share disorders with UQCRB: view

No data available for Genatlas for UQCRB Gene

Publications for UQCRB Gene

  1. A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis. (PMID: 12709789) Haut S … Slama A (Human genetics 2003) 3 4 22 58
  2. Chromosomal assignment of the gene for the ubiquinone-binding protein of human mitochondrial cytochrome bc1 complex. (PMID: 2167087) Hosokawa Y … Ozawa T (Biochemistry international 1990) 2 3 22 58
  3. Isolation of a single nuclear gene encoding human ubiquinone-binding protein in complex III of mitochondrial respiratory chain. (PMID: 2543413) Suzuki H … Ozawa T (Biochemical and biophysical research communications 1989) 2 3 4 58
  4. Cloning and sequencing of a cDNA for human mitochondrial ubiquinone-binding protein of complex III. (PMID: 3056408) Suzuki H … Ozawa T (Biochemical and biophysical research communications 1988) 2 3 4 58
  5. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson SL … O'Brien SJ (PloS one 2010) 3 44 58

Products for UQCRB Gene

  • Addgene plasmids for UQCRB

Sources for UQCRB Gene

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