SCO1 Cu-binding protein SCO1 [Saccharomyces cerevisiae S288C] - Gene

Summary

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Official Symbol: SCO1
Official Full Name: Cu-binding protein SCO1
Primary source: SGD:S000000241
Locus tag: YBR037C
See related: AllianceGenome:SGD:S000000241; FungiDB:YBR037C; VEuPathDB:YBR037C
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Saccharomyces cerevisiae S288C (strain: S288C)
Lineage: Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Also known as: PET161
Summary: Enables copper ion binding activity. Involved in cell redox homeostasis; cellular response to oxidative stress; and mitochondrial cytochrome c oxidase assembly. Located in mitochondrial inner membrane. Used to study hypertrophic cardiomyopathy and liver disease. Human ortholog(s) of this gene implicated in cytochrome-c oxidase deficiency disease; hypertrophic cardiomyopathy; mitochondrial complex IV deficiency nuclear type 2; mitochondrial complex IV deficiency nuclear type 4; and myopia. Orthologous to several human genes including SCO1 (synthesis of cytochrome C oxidase 1). [provided by Alliance of Genome Resources, Nov 2024]
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Genomic context

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See SCO1 in Genome Data Viewer

Location:: chromosome: II

Exon count:: 1

Sequence:: Chromosome: II; NC_001134.8 (310565..311452, complement)

Chromosome II - NC_001134.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_001134.8

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Rho0 cells demonstrated loss of Sco1, resulting in copper deficiency by lowering copper transporter 1 abundance, via mechanism involving increased turnover due to ubiquitination. These findings reveal that a Sco1-dependent mitochondrial signal regulates cellular cisplatin import and cytotoxicity.
Title: Low Ctr1p, due to lack of Sco1p results in lowered cisplatin uptake and mediates insensitivity of rho0 yeast to cisplatin.
HCC1 of Arabidopsis is the homologue of the Saccharomyces cerevisiae SCO1.
Title: HCC1, the Arabidopsis homologue of the yeast mitochondrial copper chaperone SCO1, is essential for embryonic development.
Thiese data suggest that the absence of Sco1p causes irreversible damage to the mitochondria.
Title: Novel insights into phenotype and mitochondrial proteome of yeast mutants lacking proteins Sco1p or Sco2p.
These studies implicate different surfaces on Sco1 for interaction or function with Cox17 and Cox2.
Title: Mapping the functional interaction of Sco1 and Cox2 in cytochrome oxidase biogenesis.
Data show that hydrogen peroxide sensitivity of sco1Delta and cox11Delta cells is abrogated by overexpression of a novel mitochondrial ATPase Afg1 that promotes the degradation of CcO mitochondrially encoded subunits.
Title: Evidence for a pro-oxidant intermediate in the assembly of cytochrome oxidase.
THe copper binding site is co-translationally formed by a transient interaction between Cox11p and this nascent cytochrome C oxidase in the intermembrane space.
Title: Evidence for the association of yeast mitochondrial ribosomes with Cox11p, a protein required for the Cu(B) site formation of cytochrome c oxidase.
Sco1 exhibits a thioredoxin-like fold, similar to that observed for human Sco1.
Title: Crystal structure of yeast Sco1.

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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 copper chaperone activity	IEA Inferred from Electronic Annotation more info
enables copper ion binding	IDA Inferred from Direct Assay more info	PubMed
enables copper ion binding	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in cell redox homeostasis	IGI Inferred from Genetic Interaction more info	PubMed
involved_in cellular response to oxidative stress	IGI Inferred from Genetic Interaction more info	PubMed
involved_in intracellular copper ion homeostasis	IEA Inferred from Electronic Annotation more info
involved_in mitochondrial cytochrome c oxidase assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrial cytochrome c oxidase assembly	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in respiratory chain complex IV assembly	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
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: Cu-binding protein SCO1

NP_009593.1

Copper-binding protein of mitochondrial inner membrane; required for cytochrome c oxidase activity and respiration; may function to deliver copper to cytochrome c oxidase; similar to thioredoxins; SCO1 has a paralog, SCO2, that arose from the whole genome duplication

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_001134.8 Reference assembly

Range

310565..311452 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_001178385.1 → NP_009593.1 TPA: Cu-binding protein SCO1 [Saccharomyces cerevisiae S288C]

See identical proteins and their annotated locations for NP_009593.1

Status: REVIEWED

UniProtKB/Swiss-Prot

D6VQ37, P23833

UniProtKB/TrEMBL

A6ZKY1, B3LNA8, B5VE03, C7GXX5, D3UED3, G2W942, N1P800

Conserved Domains (1) summary

COG1999
Location:76 → 276

Sco1; Cytochrome oxidase Cu insertion factor, SCO1/SenC/PrrC family [Posttranslational modification, protein turnover, chaperones]