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Protein

Protein SCO1 homolog, mitochondrial

Gene

Sco1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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 keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi152CopperBy similarity1
Metal bindingi156CopperBy similarity1
Metal bindingi243CopperBy similarity1

GO - Molecular functioni

GO - Biological processi

  • cell redox homeostasis Source: InterPro
  • cellular copper ion homeostasis Source: MGI
  • mitochondrial respiratory chain complex IV assembly Source: MGI
  • negative regulation of proteasomal protein catabolic process Source: MGI

Keywordsi

Molecular functionChaperone
LigandCopper, Metal-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Protein SCO1 homolog, mitochondrial
Gene namesi
Name:Sco1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:106362 Sco1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini? – 80Mitochondrial matrixBy similarity
Transmembranei81 – 98HelicalSequence analysisAdd BLAST18
Topological domaini99 – 284Mitochondrial intermembraneBy similarityAdd BLAST186

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & 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 keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi115G → S: Knockin mice hearts exhibit a severe combined COX and copper deficiency and a mislocalization of copper transporter protein CTR1 to the cytoplasm. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_0000354066? – 284Protein SCO1 homolog, mitochondrial
Transit peptidei1 – ?MitochondrionSequence analysis

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi152 ↔ 156Redox-activePROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond

Proteomic databases

EPDiQ5SUC9
MaxQBiQ5SUC9
PaxDbiQ5SUC9
PeptideAtlasiQ5SUC9
PRIDEiQ5SUC9

PTM databases

iPTMnetiQ5SUC9
PhosphoSitePlusiQ5SUC9

Expressioni

Gene expression databases

BgeeiENSMUSG00000069844
ExpressionAtlasiQ5SUC9 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 similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000090673

Structurei

3D structure databases

ProteinModelPortaliQ5SUC9
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni101 – 114Important for dimerizationBy similarityAdd BLAST14

Sequence similaritiesi

Belongs to the SCO1/2 family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2792 Eukaryota
COG1999 LUCA
GeneTreeiENSGT00390000004323
HOGENOMiHOG000258140
HOVERGENiHBG000428
InParanoidiQ5SUC9
KOiK07152
PhylomeDBiQ5SUC9
TreeFamiTF313752

Family and domain databases

CDDicd02968 SCO, 1 hit
InterProiView protein in InterPro
IPR003782 SCO1/SenC
IPR036249 Thioredoxin-like_sf
IPR013766 Thioredoxin_domain
PANTHERiPTHR12151 PTHR12151, 1 hit
PfamiView protein in Pfam
PF02630 SCO1-SenC, 1 hit
SUPFAMiSSF52833 SSF52833, 1 hit
PROSITEiView protein in PROSITE
PS51352 THIOREDOXIN_2, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q5SUC9-1 [UniParc]FASTAAdd to basket

« Hide

        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
Length:284
Mass (Da):31,617
Last modified:December 21, 2004 - v1
Checksum:i9CEC7E19F9977FF5
GO

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
CCDSiCCDS36183.1
RefSeqiNP_001035115.1, NM_001040026.1
UniGeneiMm.129731
Mm.473182

Genome annotation databases

EnsembliENSMUST00000092996; ENSMUSP00000090673; ENSMUSG00000069844
GeneIDi52892
KEGGimmu:52892
UCSCiuc007jlu.1 mouse

Similar proteinsi

Entry informationi

Entry nameiSCO1_MOUSE
AccessioniPrimary (citable) accession number: Q5SUC9
Secondary accession number(s): Q3UTD6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 25, 2008
Last sequence update: December 21, 2004
Last modified: April 25, 2018
This is version 96 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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