UniProtKB - Q5SUC9 (SCO1_MOUSE)
Protein
Protein SCO1 homolog, mitochondrial
Gene
Sco1
Organism
Mus musculus (Mouse)
Status
Functioni
Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (By similarity). Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1 (PubMed:25683716, PubMed:28973536).By similarity2 Publications
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 152 | CopperBy similarity | 1 | |
Metal bindingi | 156 | CopperBy similarity | 1 | |
Metal bindingi | 243 | CopperBy similarity | 1 |
GO - Molecular functioni
- metal ion binding Source: UniProtKB-KW
GO - Biological processi
- cellular copper ion homeostasis Source: MGI
- mitochondrial cytochrome c oxidase assembly Source: MGI
- negative regulation of proteasomal protein catabolic process Source: MGI
Keywordsi
Molecular function | Chaperone |
Ligand | Copper, Metal-binding |
Enzyme and pathway databases
Reactomei | R-MMU-5628897, TP53 Regulates Metabolic Genes R-MMU-611105, Respiratory electron transport |
Names & Taxonomyi
Protein namesi | Recommended name: Protein SCO1 homolog, mitochondrial |
Gene namesi | Name:Sco1 |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:106362, Sco1 |
Subcellular locationi
Mitochondrion
- Mitochondrion By similarity
- Mitochondrion inner membrane By similarity; Single-pass membrane protein Sequence analysis
Mitochondrion
- integral component of mitochondrial inner membrane Source: UniProtKB
- mitochondrion Source: MGI
Other locations
- myofibril Source: MGI
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | ? – 80 | Mitochondrial matrixBy similarity | ||
Transmembranei | 81 – 98 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 99 – 284 | Mitochondrial intermembraneBy similarityAdd BLAST | 186 |
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion inner membranePathology & Biotechi
Disruption phenotypei
Heart-specific and striated muscle-specific knockout mice exhibit a lethal phenotype due to a combined COX and copper deficiency that results in a dilated cardiomyopathy. Cardiac copper deficiency is caused by the mislocalisation of copper transporter CTR1 to the cytoplasm (PubMed:28973536). Liver-specific knockout mice exhibit a lethal phenotype associated with profound hepatic COX and copper deficiencies. Hepatic copper deficiency is due to reduced localization of CTR1 at the cell membrane and an enhanced proteasomal degradation of CTR1 (PubMed:25683716).2 Publications
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 115 | G → S: Knockin mice hearts exhibit a severe combined COX and copper deficiency and a mislocalization of copper transporter protein CTR1 to the cytoplasm. 1 Publication | 1 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – ? | MitochondrionSequence analysis | ||
ChainiPRO_0000354066 | ? – 284 | Protein SCO1 homolog, mitochondrial |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Disulfide bondi | 152 ↔ 156 | Redox-activePROSITE-ProRule annotation |
Keywords - PTMi
Disulfide bondProteomic databases
EPDi | Q5SUC9 |
jPOSTi | Q5SUC9 |
MaxQBi | Q5SUC9 |
PaxDbi | Q5SUC9 |
PeptideAtlasi | Q5SUC9 |
PRIDEi | Q5SUC9 |
PTM databases
iPTMneti | Q5SUC9 |
PhosphoSitePlusi | Q5SUC9 |
Expressioni
Gene expression databases
Bgeei | ENSMUSG00000069844, Expressed in heart left ventricle and 189 other tissues |
ExpressionAtlasi | Q5SUC9, baseline and differential |
Interactioni
Subunit structurei
Homodimer.
Interacts with COA6.
Found in a complex with TMEM177, COX20, COA6, MT-CO2/COX2, COX18 and SCO2.
Interacts with TMEM177 in a COX20-dependent manner.
Interacts with COX20 in a MT-CO2/COX2- and COX18-dependent manner.
Interacts with COX16.
By similarityProtein-protein interaction databases
BioGRIDi | 206870, 38 interactors |
STRINGi | 10090.ENSMUSP00000090673 |
Miscellaneous databases
RNActi | Q5SUC9, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 101 – 114 | Important for dimerizationBy similarityAdd BLAST | 14 |
Sequence similaritiesi
Belongs to the SCO1/2 family.Curated
Keywords - Domaini
Transit peptide, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG2792, Eukaryota |
GeneTreei | ENSGT00390000004323 |
HOGENOMi | CLU_050131_0_3_1 |
InParanoidi | Q5SUC9 |
OMAi | GPVTWKS |
OrthoDBi | 1462537at2759 |
PhylomeDBi | Q5SUC9 |
TreeFami | TF313752 |
Family and domain databases
CDDi | cd02968, SCO, 1 hit |
Gene3Di | 3.40.30.10, 1 hit |
InterProi | View protein in InterPro IPR003782, SCO1/SenC IPR036249, Thioredoxin-like_sf IPR013766, Thioredoxin_domain |
PANTHERi | PTHR12151, PTHR12151, 1 hit |
Pfami | View protein in Pfam PF02630, SCO1-SenC, 1 hit |
SUPFAMi | SSF52833, SSF52833, 1 hit |
PROSITEi | View protein in PROSITE PS51352, THIOREDOXIN_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
Q5SUC9-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MAALVRAAVV RSQCRQLWRL FPRGHGLRDV AERPRPEEAC SCLRSRAFSA
60 70 80 90 100
GPPPPGAGPE PKGGQAGSHR PKPGPVSWKS LALTFAIGGS LLAGMKYFKK
110 120 130 140 150
EKIEKLEKQR HRSIGKPLLG GPFSLTTHNG EPKTDKDYLG QWVLIYFGFT
160 170 180 190 200
HCPDICPEEL EKMIEVVEEI DSIPSLPNLT PLFITIDPER DTKEAIATYV
210 220 230 240 250
KEFSPKLVGL TGTKEEIDGV ARAYRVYYSP GPKDEDEDYI VDHTIIMYLI
260 270 280
GPDGEFLDYF GQNKKKAEIA GSIAAHMRSH MKKR
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketQ5SUD5 | Q5SUD5_MOUSE | Protein SCO1 homolog, mitochondrial | Sco1 | 289 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL645988 Genomic DNA No translation available. BC139008 mRNA Translation: AAI39009.1 BC139009 mRNA Translation: AAI39010.1 AK139512 mRNA Translation: BAE24044.1 |
CCDSi | CCDS36183.1 |
RefSeqi | NP_001035115.1, NM_001040026.1 |
Genome annotation databases
Ensembli | ENSMUST00000092996; ENSMUSP00000090673; ENSMUSG00000069844 |
GeneIDi | 52892 |
KEGGi | mmu:52892 |
UCSCi | uc007jlu.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL645988 Genomic DNA No translation available. BC139008 mRNA Translation: AAI39009.1 BC139009 mRNA Translation: AAI39010.1 AK139512 mRNA Translation: BAE24044.1 |
CCDSi | CCDS36183.1 |
RefSeqi | NP_001035115.1, NM_001040026.1 |
3D structure databases
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Protein-protein interaction databases
BioGRIDi | 206870, 38 interactors |
STRINGi | 10090.ENSMUSP00000090673 |
PTM databases
iPTMneti | Q5SUC9 |
PhosphoSitePlusi | Q5SUC9 |
Proteomic databases
EPDi | Q5SUC9 |
jPOSTi | Q5SUC9 |
MaxQBi | Q5SUC9 |
PaxDbi | Q5SUC9 |
PeptideAtlasi | Q5SUC9 |
PRIDEi | Q5SUC9 |
Protocols and materials databases
Antibodypediai | 12932, 152 antibodies |
Genome annotation databases
Ensembli | ENSMUST00000092996; ENSMUSP00000090673; ENSMUSG00000069844 |
GeneIDi | 52892 |
KEGGi | mmu:52892 |
UCSCi | uc007jlu.1, mouse |
Organism-specific databases
CTDi | 6341 |
MGIi | MGI:106362, Sco1 |
Phylogenomic databases
eggNOGi | KOG2792, Eukaryota |
GeneTreei | ENSGT00390000004323 |
HOGENOMi | CLU_050131_0_3_1 |
InParanoidi | Q5SUC9 |
OMAi | GPVTWKS |
OrthoDBi | 1462537at2759 |
PhylomeDBi | Q5SUC9 |
TreeFami | TF313752 |
Enzyme and pathway databases
Reactomei | R-MMU-5628897, TP53 Regulates Metabolic Genes R-MMU-611105, Respiratory electron transport |
Miscellaneous databases
BioGRID-ORCSi | 52892, 7 hits in 17 CRISPR screens |
ChiTaRSi | Kit, mouse |
PROi | PR:Q5SUC9 |
RNActi | Q5SUC9, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000069844, Expressed in heart left ventricle and 189 other tissues |
ExpressionAtlasi | Q5SUC9, baseline and differential |
Family and domain databases
CDDi | cd02968, SCO, 1 hit |
Gene3Di | 3.40.30.10, 1 hit |
InterProi | View protein in InterPro IPR003782, SCO1/SenC IPR036249, Thioredoxin-like_sf IPR013766, Thioredoxin_domain |
PANTHERi | PTHR12151, PTHR12151, 1 hit |
Pfami | View protein in Pfam PF02630, SCO1-SenC, 1 hit |
SUPFAMi | SSF52833, SSF52833, 1 hit |
PROSITEi | View protein in PROSITE PS51352, THIOREDOXIN_2, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | SCO1_MOUSE | |
Accessioni | Q5SUC9Primary (citable) accession number: Q5SUC9 Secondary accession number(s): Q3UTD6 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | November 25, 2008 |
Last sequence update: | December 21, 2004 | |
Last modified: | February 10, 2021 | |
This is version 110 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families