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UniProtKB - O00391 (QSOX1_HUMAN)

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Protein

Sulfhydryl oxidase 1

Gene

QSOX1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the oxidation of sulfhydryl groups in peptide and protein thiols to disulfides with the reduction of oxygen to hydrogen peroxide. May contribute to disulfide bond formation in a variety of secreted proteins. In fibroblasts, it may have tumor-suppressing capabilities being involved in growth regulation.6 Publications

Miscellaneous

'Quiescin Q6' means that it was the sixth clone to be found at a higher level of expression in quiescent fibroblasts.

Catalytic activityi

2 R'C(R)SH + O2 = R'C(R)S-S(R)CR' + H2O2.1 Publication

Cofactori

FAD2 PublicationsNote: Binds 1 FAD per subunit.2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei70Nucleophile1 Publication1
Active sitei73Nucleophile1 Publication1
Binding sitei401FAD1 Publication1
Binding sitei408FAD1 Publication1
Binding sitei412FAD1 Publication1
Binding sitei451FAD1 Publication1
Binding sitei455FAD1 Publication1
Binding sitei500FAD1 Publication1
Binding sitei503FAD1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi478 – 485FAD1 Publication8

GO - Molecular functioni

  • flavin-linked sulfhydryl oxidase activity Source: UniProtKB
  • protein disulfide isomerase activity Source: UniProtKB
Complete GO annotation...

GO - Biological processi

Complete GO annotation...

Keywordsi

Molecular functionOxidoreductase
LigandFAD, Flavoprotein

Enzyme and pathway databases

BRENDAi1.8.3.2. 2681.
ReactomeiR-HSA-114608. Platelet degranulation.
R-HSA-381426. Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs).
R-HSA-6798695. Neutrophil degranulation.
R-HSA-8957275. Post-translational protein phosphorylation.

Names & Taxonomyi

Protein namesi
Recommended name:
Sulfhydryl oxidase 1 (EC:1.8.3.2)
Short name:
hQSOX
Alternative name(s):
Quiescin Q6
Gene namesi
Name:QSOX1
Synonyms:QSCN6
ORF Names:UNQ2520/PRO6013
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:9756. QSOX1.

Subcellular locationi

Isoform 1 :
Isoform 2 :

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei710 – 730HelicalSequence analysisAdd BLAST21

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane, Secreted

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi70C → S: Reduces activity by 93%. 1 Publication1
Mutagenesisi73C → S: Reduces activity by 93%. 1 Publication1
Mutagenesisi449C → S: Reduces activity by 96%. 1 Publication1
Mutagenesisi452C → S: Loss of activity. 1 Publication1
Mutagenesisi509C → S: No effect. Reduces activity by 70%; when associated with S-512. 1 Publication1
Mutagenesisi512C → S: Reduces activity by 40%. Reduces activity by 70%; when associated with S-509. 1 Publication1

Organism-specific databases

DisGeNETi5768.
OpenTargetsiENSG00000116260.
PharmGKBiPA162400559.

Polymorphism and mutation databases

BioMutaiQSOX1.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 29Sequence analysisAdd BLAST29
ChainiPRO_000024953330 – 747Sulfhydryl oxidase 1Add BLAST718

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi70 ↔ 73Redox-active1 Publication
Glycosylationi130N-linked (GlcNAc...) (complex) asparagine2 Publications1
Glycosylationi243N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi393 ↔ 405PROSITE-ProRule annotation1 Publication
Modified residuei426Phosphoserine; by FAM20C1 Publication1
Disulfide bondi449 ↔ 452PROSITE-ProRule annotation1 Publication
Disulfide bondi509 ↔ 512PROSITE-ProRule annotation1 Publication
Glycosylationi575N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

EPDiO00391.
MaxQBiO00391.
PaxDbiO00391.
PeptideAtlasiO00391.
PRIDEiO00391.

PTM databases

iPTMnetiO00391.
PhosphoSitePlusiO00391.

Expressioni

Tissue specificityi

Expressed in heart, placenta, lung, liver, skeletal muscle, pancreas and very weakly in brain and kidney.2 Publications

Inductioni

Induced in quiescent cells just as fibroblasts begin to leave the proliferative cycle and enter quiescence.3 Publications

Gene expression databases

BgeeiENSG00000116260.
CleanExiHS_QSOX1.
ExpressionAtlasiO00391. baseline and differential.
GenevisibleiO00391. HS.

Organism-specific databases

HPAiHPA042127.
HPA056243.

Interactioni

Subunit structurei

Monomer.2 Publications

Protein-protein interaction databases

BioGridi111734. 15 interactors.
IntActiO00391. 6 interactors.
MINTiMINT-2862351.
STRINGi9606.ENSP00000356574.

Structurei

Secondary structure

1747
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi40 – 45Combined sources6
Turni47 – 49Combined sources3
Helixi50 – 54Combined sources5
Beta strandi58 – 66Combined sources9
Helixi71 – 86Combined sources16
Helixi88 – 90Combined sources3
Turni91 – 93Combined sources3
Beta strandi94 – 100Combined sources7
Turni104 – 106Combined sources3
Helixi107 – 112Combined sources6
Beta strandi117 – 124Combined sources8
Beta strandi130 – 132Combined sources3
Beta strandi134 – 136Combined sources3
Helixi143 – 155Combined sources13
Helixi173 – 177Combined sources5
Helixi179 – 182Combined sources4
Beta strandi186 – 193Combined sources8
Helixi199 – 206Combined sources8
Turni207 – 209Combined sources3
Beta strandi213 – 219Combined sources7
Helixi223 – 229Combined sources7
Beta strandi234 – 241Combined sources8
Beta strandi246 – 248Combined sources3
Beta strandi252 – 255Combined sources4
Helixi256 – 264Combined sources9
Beta strandi298 – 300Combined sources3
Helixi301 – 313Combined sources13
Helixi316 – 318Combined sources3
Beta strandi320 – 323Combined sources4
Helixi324 – 340Combined sources17
Helixi345 – 360Combined sources16
Beta strandi363 – 367Combined sources5
Helixi368 – 377Combined sources10
Helixi380 – 383Combined sources4
Beta strandi400 – 402Combined sources3
Helixi403 – 419Combined sources17
Helixi426 – 431Combined sources6
Helixi432 – 434Combined sources3
Helixi435 – 446Combined sources12
Helixi450 – 463Combined sources14
Helixi464 – 466Combined sources3
Helixi470 – 488Combined sources19
Beta strandi502 – 504Combined sources3
Turni506 – 508Combined sources3
Helixi510 – 512Combined sources3
Beta strandi517 – 519Combined sources3
Helixi524 – 534Combined sources11
Helixi537 – 539Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3LLIX-ray2.05A286-546[»]
3LLKX-ray2.00A/B/C286-546[»]
3Q6OX-ray2.05A33-272[»]
4IJ3X-ray2.70A33-272[»]
ProteinModelPortaliO00391.
SMRiO00391.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiO00391.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini36 – 156ThioredoxinPROSITE-ProRule annotationAdd BLAST121
Domaini396 – 503ERV/ALR sulfhydryl oxidasePROSITE-ProRule annotationAdd BLAST108

Sequence similaritiesi

Belongs to the quiescin-sulfhydryl oxidase (QSOX) family.Curated

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1731. Eukaryota.
ENOG410XVJT. LUCA.
GeneTreeiENSGT00390000008045.
HOGENOMiHOG000231631.
HOVERGENiHBG080360.
InParanoidiO00391.
KOiK10758.
OMAiMMVLAFK.
OrthoDBiEOG091G038J.
PhylomeDBiO00391.
TreeFamiTF316749.

Family and domain databases

Gene3Di1.20.120.310. 1 hit.
InterProiView protein in InterPro
IPR017905. ERV/ALR_sulphydryl_oxidase.
IPR012336. Thioredoxin-like_fold.
IPR013766. Thioredoxin_domain.
PfamiView protein in Pfam
PF04777. Evr1_Alr. 1 hit.
PF00085. Thioredoxin. 1 hit.
SUPFAMiSSF52833. SSF52833. 1 hit.
SSF69000. SSF69000. 1 hit.
PROSITEiView protein in PROSITE
PS51324. ERV_ALR. 1 hit.
PS51352. THIOREDOXIN_2. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: O00391-1) [UniParc]FASTAAdd to basket
Also known as: a, QSOX-L

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MRRCNSGSGP PPSLLLLLLW LLAVPGANAA PRSALYSPSD PLTLLQADTV 
        60         70         80         90        100
RGAVLGSRSA WAVEFFASWC GHCIAFAPTW KALAEDVKAW RPALYLAALD 
       110        120        130        140        150
CAEETNSAVC RDFNIPGFPT VRFFKAFTKN GSGAVFPVAG ADVQTLRERL 
       160        170        180        190        200
IDALESHHDT WPPACPPLEP AKLEEIDGFF ARNNEEYLAL IFEKGGSYLG 
       210        220        230        240        250
REVALDLSQH KGVAVRRVLN TEANVVRKFG VTDFPSCYLL FRNGSVSRVP 
       260        270        280        290        300
VLMESRSFYT AYLQRLSGLT REAAQTTVAP TTANKIAPTV WKLADRSKIY 
       310        320        330        340        350
MADLESALHY ILRIEVGRFP VLEGQRLVAL KKFVAVLAKY FPGRPLVQNF 
       360        370        380        390        400
LHSVNEWLKR QKRNKIPYSF FKTALDDRKE GAVLAKKVNW IGCQGSEPHF 
       410        420        430        440        450
RGFPCSLWVL FHFLTVQAAR QNVDHSQEAA KAKEVLPAIR GYVHYFFGCR 
       460        470        480        490        500
DCASHFEQMA AASMHRVGSP NAAVLWLWSS HNRVNARLAG APSEDPQFPK 
       510        520        530        540        550
VQWPPRELCS ACHNERLDVP VWDVEATLNF LKAHFSPSNI ILDFPAAGSA 
       560        570        580        590        600
ARRDVQNVAA APELAMGALE LESRNSTLDP GKPEMMKSPT NTTPHVPAEG 
       610        620        630        640        650
PEASRPPKLH PGLRAAPGQE PPEHMAELQR NEQEQPLGQW HLSKRDTGAA 
       660        670        680        690        700
LLAESRAEKN RLWGPLEVRR VGRSSKQLVD IPEGQLEARA GRGRGQWLQV 
       710        720        730        740 
LGGGFSYLDI SLCVGLYSLS FMGLLAMYTY FQAKIRALKG HAGHPAA
Length:747
Mass (Da):82,578
Last modified:June 1, 2001 - v3
Checksum:i52639D09A50E00C5
GO
Isoform 2 (identifier: O00391-2) [UniParc]FASTAAdd to basket
Also known as: b, QSOX-S

The sequence of this isoform differs from the canonical sequence as follows:
     603-604: AS → LI
     605-747: Missing.

Show »
Length:604
Mass (Da):66,861
Checksum:iB557EE4D1525E7FB
GO

Sequence cautioni

The sequence BAD92517 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_027429114N → S. Corresponds to variant dbSNP:rs3894211Ensembl.1
Natural variantiVAR_027430200G → A3 PublicationsCorresponds to variant dbSNP:rs17855475Ensembl.1
Natural variantiVAR_027431256R → M. Corresponds to variant dbSNP:rs4360492Ensembl.1
Natural variantiVAR_027432294A → S. Corresponds to variant dbSNP:rs2278943Ensembl.1
Natural variantiVAR_027433444H → R1 PublicationCorresponds to variant dbSNP:rs12371Ensembl.1
Natural variantiVAR_027434591N → H. Corresponds to variant dbSNP:rs3738115Ensembl.1
Natural variantiVAR_053652605R → P. Corresponds to variant dbSNP:rs16855466Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_020489603 – 604AS → LI in isoform 2. 2 Publications2
Alternative sequenceiVSP_020490605 – 747Missing in isoform 2. 2 PublicationsAdd BLAST143

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U97276 mRNA. Translation: AAC09010.2.
AF361868 mRNA. Translation: AAM00263.1.
AY358941 mRNA. Translation: AAQ89300.1.
AB209280 mRNA. Translation: BAD92517.1. Different initiation.
AL390718 Genomic DNA. Translation: CAI14838.1.
AL390718 Genomic DNA. Translation: CAI14839.1.
BC017692 mRNA. Translation: AAH17692.1.
BC100023 mRNA. Translation: AAI00024.1.
CCDSiCCDS1337.1. [O00391-1]
CCDS30950.1. [O00391-2]
RefSeqiNP_001004128.1. NM_001004128.2. [O00391-2]
NP_002817.2. NM_002826.4. [O00391-1]
UniGeneiHs.744925.

Genome annotation databases

EnsembliENST00000367600; ENSP00000356572; ENSG00000116260. [O00391-2]
ENST00000367602; ENSP00000356574; ENSG00000116260. [O00391-1]
GeneIDi5768.
KEGGihsa:5768.
UCSCiuc001gny.4. human. [O00391-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.

Entry informationi

Entry nameiQSOX1_HUMAN
AccessioniPrimary (citable) accession number: O00391
Secondary accession number(s): Q59G29
, Q5T2X0, Q8TDL6, Q8WVP4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 19, 2006
Last sequence update: June 1, 2001
Last modified: July 5, 2017
This is version 152 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families