BCS1 bifunctional AAA family ATPase chaperone/translocase BCS1 [Saccharomyces cerevisiae S288C] - Gene

Summary

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Gene symbol: BCS1
Gene description: bifunctional AAA family ATPase chaperone/translocase BCS1
Primary source: FungiDB:YDR375C
Locus tag: YDR375C
See related: SGD:S000002783; AllianceGenome:SGD:S000002783; VEuPathDB:YDR375C
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Saccharomyces cerevisiae S288C (strain: S288C)
Lineage: Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Summary: Enables ATPase-coupled transmembrane transporter activity and protein transmembrane transporter activity. Involved in chaperone-mediated protein complex assembly; mitochondrial respiratory chain complex III assembly; and protein insertion into mitochondrial inner membrane from matrix. Located in mitochondrial inner membrane. Used to study mitochondrial complex III deficiency nuclear type 1. 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, Apr 2022]
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Genomic context

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See BCS1 in Genome Data Viewer

Location:: chromosome: IV

Exon count:: 1

Sequence:: Chromosome: IV; NC_001136.10 (1225166..1226536, complement)

Chromosome IV - NC_001136.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_001136.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Structural snapshots of the cellular folded protein translocation machinery Bcs1.
Title: Structural snapshots of the cellular folded protein translocation machinery Bcs1.
This study presents the structure of Saccharomyces cerevisiae Bcs1, an AAA-ATPase of the inner mitochondrial membrane. Bcs1 facilitates the translocation of the Rieske protein, Rip1, which requires folding and incorporation of a 2Fe-2S cluster before translocation and subsequent integration into the bc1 complex.
Title: Structure of the Bcs1 AAA-ATPase suggests an airlock-like translocation mechanism for folded proteins.
We found that the absence of non-essential translation factors Rrf1 or Mif3, which act at the recycling/initiation steps, also compensates for the respiratory deficiency of yeast bcs1 mutations
Title: Chemicals or mutations that target mitochondrial translation can rescue the respiratory deficiency of yeast bcs1 mutants.
The ATP dependency of the protein Bcs1 is not just a requirement for its chaperon activity but also a way to couple the rate of complex III biogenesis to the energy-transducing activity of mitochondria.
Title: The energetic state of mitochondria modulates complex III biogenesis through the ATP-dependent activity of Bcs1.
The evolutionarily-conserved short alpha helix of Bcs1 in the intermembrane space is an essential element for the chaperone function.
Title: A conserved α helix of Bcs1, a mitochondrial AAA chaperone, is required for the Respiratory Complex III maturation.
The oligomeric AAA-ATPase Bcs1 is involved in export of the folded Fe-S domain of Rip1 across the inner membrane and insertion of its transmembrane segment into an assembly intermediate of the cytochrome bc(1) complex.
Title: A pathway of protein translocation in mitochondria mediated by the AAA-ATPase Bcs1.
residues located at the junction between the Bcs1p-specific and the AAA domains are important for the activity and stability of the protein, and the residue F342 is important for interactions with other partners or substrate proteins.
Title: Functional analysis of yeast bcs1 mutants highlights the role of Bcs1p-specific amino acids in the AAA domain.

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Homology

Orthologs from OrthoDB

Gene Ontology Provided by SGD

Function	Evidence Code	Pubs
enables ATP binding	IEA Inferred from Electronic Annotation more info
enables ATP hydrolysis activity	IEA Inferred from Electronic Annotation more info
enables ATPase-coupled transmembrane transporter activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables protein transmembrane transporter activity	IMP Inferred from Mutant Phenotype more info	PubMed

Process	Evidence Code	Pubs
involved_in chaperone-mediated protein complex assembly	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in mitochondrial respiratory chain complex III assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrial respiratory chain complex III assembly	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein insertion into mitochondrial inner membrane from matrix	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein insertion into mitochondrial inner membrane from matrix	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein insertion into mitochondrial inner membrane from matrix	IPI Inferred from Physical Interaction more info	PubMed
involved_in protein transmembrane transport	IEA Inferred from Electronic Annotation more info
involved_in transmembrane transport	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
is_active_in mitochondrial inner membrane	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial inner membrane	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: bifunctional AAA family ATPase chaperone/translocase BCS1

NP_010663.1

Protein translocase and chaperone required for Complex III assembly; member of the AAA ATPase family; forms a homo-oligomeric complex in the mitochondrial inner membrane that translocates the C-terminal domain of Rip1p from the matrix across the inner membrane and delivers it to an assembly intermediate of respiratory Complex III; also required for assembly of the Qcr10p subunit; mutation is functionally complemented by human homolog BCS1L, linked to neonatal diseases

NCBI Reference Sequences (RefSeq)

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Genome Annotation

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

Reference assembly

Genomic

NC_001136.10 Reference assembly

Range

1225166..1226536 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_001180683.1 → NP_010663.1 TPA: bifunctional AAA family ATPase chaperone/translocase BCS1 [Saccharomyces cerevisiae S288C]

See identical proteins and their annotated locations for NP_010663.1

Status: REVIEWED

UniProtKB/Swiss-Prot

D6VT07, P32839, Q06404

UniProtKB/TrEMBL

A0A6A5PZ31, A6ZYX4, B5VGQ5, C7GMG1, C8Z5T8, G2WBB2, N1P5E7

Conserved Domains (1) summary

COG0465
Location:1 → 456

HflB; ATP-dependent Zn proteases [Posttranslational modification, protein turnover, chaperones]