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UQCR-C1 Ubiquinol-cytochrome c reductase core protein 1 [ Drosophila melanogaster (fruit fly) ]

Gene ID: 41800, updated on 6-Jul-2026
Official Symbol
UQCR-C1provided by FlyBase
Official Full Name
Ubiquinol-cytochrome c reductase core protein 1provided by FlyBase
Primary source
FLYBASE:FBgn0038271
Locus tag
Dmel_CG3731
See related
AllianceGenome:FB:FBgn0038271
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Drosophila melanogaster
Lineage
Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
Also known as
CG 3731; CG3731; Dmel\CG3731; Mpp; MPPB; UCR1; UQCR-cp1; uqcrc1
Summary
Predicted to enable metalloendopeptidase activity. Predicted to be involved in protein processing involved in protein targeting to mitochondrion. Located in mitochondrion. Is expressed in several structures, including adult head; adult heart; dorsal pharyngeal muscle cell; embryonic/larval midgut; and embryonic/larval midgut primordium. Used to study late onset Parkinson's disease. Human ortholog(s) of this gene implicated in multiple mitochondrial dysfunctions syndrome 6. Orthologous to human PMPCB (peptidase, mitochondrial processing subunit beta). [provided by Alliance of Genome Resources, Jul 2025]
Orthologs
all
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See UQCR-C1 in Genome Data Viewer
Location:
88D6-88D6; 3-56 cM
Exon count:
7
Annotation release Status Assembly Chr Location
Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 3R NT_033777.3 (14890231..14892387)
Release 5.57 previous assembly Release 5 (GCF_000001215.2) 3R NT_033777.2 (10715953..10718109)

Chromosome 3R - NT_033777.3Genomic Context describing neighboring genes Neighboring gene uncharacterized protein Neighboring gene uncharacterized protein Neighboring gene Cyclin C Neighboring gene Diphthamide biosynthesis 2 Neighboring gene uncharacterized protein Neighboring gene Nucleoporin 93kD-2

Go to nucleotide: Graphics FASTA GenBank

Related articles in PubMed

  1. Mitochondrial UQCRC1 mutations cause autosomal dominant parkinsonism with polyneuropathy. Lin CH, et al. Brain, 2020 Dec 5. PMID 33141179, Free PMC Article
  2. Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation. Fernandez-Ayala DJ, et al. Cell Metab, 2009 May. PMID 19416715
  3. Mitochondrial processing peptidases. Gakh O, et al. Biochim Biophys Acta, 2002 Sep 2. PMID 12191769
  4. UQCRC1 engages cytochrome c for neuronal apoptotic cell death. Hung YC, et al. Cell Rep, 2021 Sep 21. PMID 34551295
  5. Practical Recommendations for the Use of the GeneSwitch Gal4 System to Knock-Down Genes in Drosophila melanogaster. Scialo F, et al. PLoS One, 2016. PMID 27570965, Free PMC Article

See all (87) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. UQCRC1 engages cytochrome c for neuronal apoptotic cell death.
    Title: UQCRC1 engages cytochrome c for neuronal apoptotic cell death.
Submit: New GeneRIF Correction
Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Gene Ontology Provided by FlyBase

Function Evidence Code Pubs
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables metalloendopeptidase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables metalloendopeptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables metalloendopeptidase activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables signal peptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables signal peptidase activity NAS
Non-traceable Author Statement
more info
 PubMed 
Process Evidence Code Pubs
involved_in mitochondrial electron transport, ubiquinol to cytochrome c NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in protein localization to mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in protein processing ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in protein processing NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in proteolysis IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
part_of mitochondrial inner membrane ISS
Inferred from Sequence or Structural Similarity
more info
  
is_active_in mitochondrial matrix IC
Inferred by Curator
more info
  
located_in mitochondrial matrix IEA
Inferred from Electronic Annotation
more info
  
part_of mitochondrial processing peptidase complex IBA
Inferred from Biological aspect of Ancestor
more info
  
part_of mitochondrial processing peptidase complex ISS
Inferred from Sequence or Structural Similarity
more info
  
part_of mitochondrial processing peptidase complex NAS
Non-traceable Author Statement
more info
 PubMed 
located_in mitochondrion HDA PubMed 
located_in mitochondrion IEA
Inferred from Electronic Annotation
more info
  
part_of respiratory chain complex III ISS
Inferred from Sequence or Structural Similarity
more info
  
part_of respiratory chain complex III NAS
Non-traceable Author Statement
more info
 PubMed 
Preferred Names
Ubiquinol-cytochrome c reductase core protein 1
Names
CG3731-PA
CG3731-PB
UQCR-C1-PA
UQCR-C1-PB
Ubiquinol-cytochrome C reductase complex core protein I
Ubiquinol-cytochrome c oxidoreductase core protein 1
mitochondrial processing peptidase beta subunit
mitochondrial-processing protease
NP_650401.1
NP_731954.1

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

  1. NT_033777.3 Reference assembly

    Range
    14890231..14892387
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_169607.2 → NP_731954.1  Ubiquinol-cytochrome c reductase core protein 1, isoform A

    See identical proteins and their annotated locations for NP_731954.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q7K401, Q95U87, Q9VFF0
    Related
    FBpp0082459
    Conserved Domains (1) summary
    COG0612
    Location:35 → 459
    PqqL; Predicted Zn-dependent peptidase [General function prediction only]

  2. NM_142144.3 → NP_650401.1  Ubiquinol-cytochrome c reductase core protein 1, isoform B

    See identical proteins and their annotated locations for NP_650401.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q7K401, Q95U87, Q9VFF0
    Related
    FBpp0082460
    Conserved Domains (1) summary
    COG0612
    Location:35 → 459
    PqqL; Predicted Zn-dependent peptidase [General function prediction only]
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9VFF0 Q9VFF0

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