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Bcs1l BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone [ Rattus norvegicus (Norway rat) ]

Gene ID: 301514, updated on 4-Jan-2025

Summary

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Official Symbol
Bcs1lprovided by RGD
Official Full Name
BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperoneprovided by RGD
Primary source
RGD:1359658
See related
EnsemblRapid:ENSRNOG00000016754 AllianceGenome:RGD:1359658
Gene type
protein coding
RefSeq status
PROVISIONAL
Organism
Rattus norvegicus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
Summary
Predicted to enable ATP hydrolysis activity. Predicted to be involved in mitochondrial cytochrome c oxidase assembly and mitochondrial respiratory chain complex I assembly. Predicted to act upstream of or within mitochondrial respiratory chain complex III assembly. Predicted to be located in mitochondrion. Human ortholog(s) of this gene implicated in Bjornstad syndrome; GRACILE syndrome; and mitochondrial complex III deficiency nuclear type 1. Orthologous to human BCS1L (BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone). [provided by Alliance of Genome Resources, Jan 2025]
Expression
Biased expression in Heart (RPKM 83.8), Muscle (RPKM 61.9) and 9 other tissues See more
Orthologs
human mouse all
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Genomic context

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See Bcs1l in Genome Data Viewer
Location:
9q33
Exon count:
8
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCr8 (GCF_036323735.1) 9 NC_086027.1 (83614045..83618052)
RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 9 NC_051344.1 (76164925..76168940)
106 previous assembly Rnor_6.0 (GCF_000001895.5) 9 NC_005108.4 (81868158..81872201)

Chromosome 9 - NC_086027.1Genomic Context describing neighboring genes Neighboring gene ubiquitin specific peptidase 37 Neighboring gene CCR4-NOT transcription complex subunit 9 Neighboring gene phospholipase C, delta 4 Neighboring gene zinc finger protein 142 Neighboring gene ring finger protein 25 Neighboring gene serine/threonine kinase 36 Neighboring gene uncharacterized LOC134480595

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
  • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
  • BioProject: PRJNA238328
  • Publication: PMID 24510058
  • Analysis date: Mon Jun 6 17:44:12 2016

Bibliography

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Related articles in PubMed

  1. The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy. Levéen P, et al. Hepatology, 2011 Feb. PMID 21274865
  2. GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Visapää I, et al. Am J Hum Genet, 2002 Oct. PMID 12215968, Free PMC Article
  3. A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure. de Lonlay P, et al. Nat Genet, 2001 Sep. PMID 11528392
  4. Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L. Tamai S, et al. J Cell Sci, 2008 Aug 1. PMID 18628306
  5. A mitochondrial protein compendium elucidates complex I disease biology. Pagliarini DJ, et al. Cell, 2008 Jul 11. PMID 18614015, Free PMC Article

See all (7) citations in PubMed

GeneRIFs: Gene References Into Functions

General gene information

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Markers

Homology

Clone Names

  • MGC94495

Gene Ontology Provided by RGD

Function Evidence Code Pubs
enables ATP binding IEA
Inferred from Electronic Annotation
more info
  
enables ATP hydrolysis activity ISO
Inferred from Sequence Orthology
more info
  
Process Evidence Code Pubs
involved_in biological_process ND
No biological Data available
more info
  
involved_in mitochondrial cytochrome c oxidase assembly ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrial respiratory chain complex I assembly ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within mitochondrial respiratory chain complex III assembly ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrial respiratory chain complex III assembly ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrion organization ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
mitochondrial chaperone BCS1
Names
BC1 (ubiquinol-cytochrome c reductase) synthesis-like

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001007666.1 → NP_001007667.1  mitochondrial chaperone BCS1

    See identical proteins and their annotated locations for NP_001007667.1

    Status: PROVISIONAL

    Source sequence(s)
    BC083660
    UniProtKB/TrEMBL
    A0A8I6G9R3, A6JVV4, F7F265, Q5XIM0
    Related
    ENSRNOP00000022632.3, ENSRNOT00000022632.7
    Conserved Domains (2) summary
    smart01024
    Location:24 → 191
    BCS1_N; This domain is found at the N terminal of the mitochondrial ATPase BCS1. It encodes the import and intramitochondrial sorting for the protein
    cd19510
    Location:201 → 353
    RecA-like_BCS1; Mitochondrial chaperone BCS1

RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

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

Reference GRCr8

Genomic

  1. NC_086027.1 Reference GRCr8

    Range
    83614045..83618052
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_017596370.3 → XP_017451859.1  mitochondrial chaperone BCS1 isoform X1

    UniProtKB/TrEMBL
    A0A8I6G9R3, A6JVV4, F7F265, Q5XIM0
    Conserved Domains (2) summary
    smart01024
    Location:24 → 191
    BCS1_N; This domain is found at the N terminal of the mitochondrial ATPase BCS1. It encodes the import and intramitochondrial sorting for the protein
    cd19510
    Location:201 → 353
    RecA-like_BCS1; Mitochondrial chaperone BCS1
Nucleotide Protein Strain
Heading Accession and Version

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