Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol UQCRFS1 contributors: mct - updated : 21-01-2020
HGNC name ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
HGNC id 12587
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Digestivemouthtongue  highly
Reproductivefemale systemovary  highly
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
rieske (2Fe-2s) domain
HOMOLOGY
Homologene
FAMILY UQCR11/QCR10 family
CATEGORY enzyme , transport carrier
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,inner
intracellular,cytoplasm,organelle,membrane
basic FUNCTION
  • function as an iron-sulfur protein binding factor
  • appears to be involved in development of more aggressive phenotype of breast cancer
  • may be likely part of mitochondrial permeability transition(MPT) pores and dephosphorylation of UQCRFS1 may play a role in regulation of the MPT
  • is essential for Complex III (CIII) activity
  • UQCRFS1 is the last incorporated mitochondrial complex III (cIII) subunit, and its presence is essential for enzymatic activity
  • crucial role of UQCRFS1 for the catalytic function of CIII
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • component of the ubiquinol-cytochrome C reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • works as a human Rieske Fe-S protein (UQCRFS1) chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane
  • HSCB, essential for Fe-S cluster biogenesis of SDHB, directly binds LYRM7, formerly described as a chaperone that stabilizes UQCRFS1 prior to its insertion into CIII
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) C3MTND1
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • cell-specific Uqcrfs1 knockout in regulatory T cells in mice led to an early-onset fatal inflammatory disease without any effect on the number of regulatory T cells