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Protein

Squalene monooxygenase

Gene

SQLE

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

Functioni

Catalyzes the first oxygenation step in sterol biosynthesis and is suggested to be one of the rate-limiting enzymes in this pathway.

Catalytic activityi

Squalene + [reduced NADPH--hemoprotein reductase] + O2 = (3S)-2,3-epoxy-2,3-dihydrosqualene + [oxidized NADPH--hemoprotein reductase] + H2O.

Cofactori

Pathwayi: lanosterol biosynthesis

This protein is involved in step 2 of the subpathway that synthesizes lanosterol from farnesyl diphosphate.
Proteins known to be involved in the 3 steps of the subpathway in this organism are:
  1. Squalene synthase (FDFT1)
  2. Squalene monooxygenase (SQLE)
  3. Lanosterol synthase (LSS)
This subpathway is part of the pathway lanosterol biosynthesis, which is itself part of Terpene metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes lanosterol from farnesyl diphosphate, the pathway lanosterol biosynthesis and in Terpene metabolism.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi126 – 153FADSequence analysisAdd BLAST28

GO - Molecular functioni

  • flavin adenine dinucleotide binding Source: InterPro
  • squalene monooxygenase activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

FAD, Flavoprotein, NADP

Enzyme and pathway databases

BioCyciMetaCyc:HS02595-MONOMER.
ZFISH:HS02595-MONOMER.
ReactomeiR-HSA-191273. Cholesterol biosynthesis.
R-HSA-2426168. Activation of gene expression by SREBF (SREBP).
SABIO-RKQ14534.
UniPathwayiUPA00767; UER00752.

Chemistry databases

SwissLipidsiSLP:000001243.

Names & Taxonomyi

Protein namesi
Recommended name:
Squalene monooxygenase (EC:1.14.14.17)
Alternative name(s):
Squalene epoxidase
Short name:
SE
Gene namesi
Name:SQLE
Synonyms:ERG1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:11279. SQLE.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei20 – 40HelicalSequence analysisAdd BLAST21
Transmembranei61 – 81HelicalSequence analysisAdd BLAST21
Transmembranei123 – 143HelicalSequence analysisAdd BLAST21
Transmembranei546 – 566HelicalSequence analysisAdd BLAST21

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane, Microsome

Pathology & Biotechi

Organism-specific databases

DisGeNETi6713.
OpenTargetsiENSG00000104549.
PharmGKBiPA36108.

Chemistry databases

ChEMBLiCHEMBL3592.
DrugBankiDB01091. Butenafine.
DB00735. Naftifine.
DB00857. Terbinafine.
GuidetoPHARMACOLOGYi2433.

Polymorphism and mutation databases

BioMutaiSQLE.
DMDMi296439362.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002098381 – 574Squalene monooxygenaseAdd BLAST574

Proteomic databases

EPDiQ14534.
PaxDbiQ14534.
PeptideAtlasiQ14534.
PRIDEiQ14534.

PTM databases

iPTMnetiQ14534.
PhosphoSitePlusiQ14534.
SwissPalmiQ14534.

Expressioni

Gene expression databases

BgeeiENSG00000104549.
CleanExiHS_SQLE.
ExpressionAtlasiQ14534. baseline and differential.
GenevisibleiQ14534. HS.

Organism-specific databases

HPAiHPA018038.
HPA020762.

Interactioni

Subunit structurei

May form a complex with squalene synthase.

Protein-protein interaction databases

BioGridi112591. 15 interactors.
IntActiQ14534. 4 interactors.
STRINGi9606.ENSP00000265896.

Chemistry databases

BindingDBiQ14534.

Structurei

3D structure databases

ProteinModelPortaliQ14534.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the squalene monooxygenase family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1298. Eukaryota.
COG0654. LUCA.
GeneTreeiENSGT00390000011759.
HOGENOMiHOG000174713.
HOVERGENiHBG005603.
InParanoidiQ14534.
KOiK00511.
OMAiQNSFYRS.
OrthoDBiEOG091G03XY.
PhylomeDBiQ14534.
TreeFamiTF331056.

Family and domain databases

Gene3Di3.50.50.60. 2 hits.
InterProiIPR023753. FAD/NAD-binding_dom.
IPR013698. Squalene_epoxidase.
[Graphical view]
PfamiPF08491. SE. 1 hit.
[Graphical view]
SUPFAMiSSF51905. SSF51905. 2 hits.

Sequencei

Sequence statusi: Complete.

Q14534-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MWTFLGIATF TYFYKKFGDF ITLANREVLL CVLVFLSLGL VLSYRCRHRN
60 70 80 90 100
GGLLGRQQSG SQFALFSDIL SGLPFIGFFW AKSPPESENK EQLEARRRRK
110 120 130 140 150
GTNISETSLI GTAACTSTSS QNDPEVIIVG AGVLGSALAA VLSRDGRKVT
160 170 180 190 200
VIERDLKEPD RIVGEFLQPG GYHVLKDLGL GDTVEGLDAQ VVNGYMIHDQ
210 220 230 240 250
ESKSEVQIPY PLSENNQVQS GRAFHHGRFI MSLRKAAMAE PNAKFIEGVV
260 270 280 290 300
LQLLEEDDVV MGVQYKDKET GDIKELHAPL TVVADGLFSK FRKSLVSNKV
310 320 330 340 350
SVSSHFVGFL MKNAPQFKAN HAELILANPS PVLIYQISSS ETRVLVDIRG
360 370 380 390 400
EMPRNLREYM VEKIYPQIPD HLKEPFLEAT DNSHLRSMPA SFLPPSSVKK
410 420 430 440 450
RGVLLLGDAY NMRHPLTGGG MTVAFKDIKL WRKLLKGIPD LYDDAAIFEA
460 470 480 490 500
KKSFYWARKT SHSFVVNILA QALYELFSAT DDSLHQLRKA CFLYFKLGGE
510 520 530 540 550
CVAGPVGLLS VLSPNPLVLI GHFFAVAIYA VYFCFKSEPW ITKPRALLSS
560 570
GAVLYKACSV IFPLIYSEMK YMVH
Length:574
Mass (Da):63,923
Last modified:May 18, 2010 - v3
Checksum:i6957F5CF1F1624C8
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti43S → F in BX647400 (PubMed:17974005).Curated1
Sequence conflicti58Q → R in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti94E → G in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti139A → V in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti247E → G in BAA11209 (PubMed:8626488).Curated1
Sequence conflicti334I → T in BX647400 (PubMed:17974005).Curated1
Sequence conflicti336Q → R in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti379 – 380AT → VA in BX647400 (PubMed:17974005).Curated2
Sequence conflicti389P → L in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti451K → N in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti518V → A in BAA22372 (PubMed:9286711).Curated1
Sequence conflicti551G → S in BAA22372 (PubMed:9286711).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D78130 mRNA. Translation: BAA22372.1.
BX647400 mRNA. No translation available.
AC009908 Genomic DNA. No translation available.
FJ695197 Genomic DNA. No translation available.
D78129 mRNA. Translation: BAA11209.1.
CCDSiCCDS47918.1.
RefSeqiNP_003120.2. NM_003129.3.
UniGeneiHs.71465.

Genome annotation databases

EnsembliENST00000265896; ENSP00000265896; ENSG00000104549.
GeneIDi6713.
KEGGihsa:6713.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D78130 mRNA. Translation: BAA22372.1.
BX647400 mRNA. No translation available.
AC009908 Genomic DNA. No translation available.
FJ695197 Genomic DNA. No translation available.
D78129 mRNA. Translation: BAA11209.1.
CCDSiCCDS47918.1.
RefSeqiNP_003120.2. NM_003129.3.
UniGeneiHs.71465.

3D structure databases

ProteinModelPortaliQ14534.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112591. 15 interactors.
IntActiQ14534. 4 interactors.
STRINGi9606.ENSP00000265896.

Chemistry databases

BindingDBiQ14534.
ChEMBLiCHEMBL3592.
DrugBankiDB01091. Butenafine.
DB00735. Naftifine.
DB00857. Terbinafine.
GuidetoPHARMACOLOGYi2433.
SwissLipidsiSLP:000001243.

PTM databases

iPTMnetiQ14534.
PhosphoSitePlusiQ14534.
SwissPalmiQ14534.

Polymorphism and mutation databases

BioMutaiSQLE.
DMDMi296439362.

Proteomic databases

EPDiQ14534.
PaxDbiQ14534.
PeptideAtlasiQ14534.
PRIDEiQ14534.

Protocols and materials databases

DNASUi6713.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000265896; ENSP00000265896; ENSG00000104549.
GeneIDi6713.
KEGGihsa:6713.

Organism-specific databases

CTDi6713.
DisGeNETi6713.
GeneCardsiSQLE.
H-InvDBHIX0007779.
HGNCiHGNC:11279. SQLE.
HPAiHPA018038.
HPA020762.
MIMi602019. gene.
neXtProtiNX_Q14534.
OpenTargetsiENSG00000104549.
PharmGKBiPA36108.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1298. Eukaryota.
COG0654. LUCA.
GeneTreeiENSGT00390000011759.
HOGENOMiHOG000174713.
HOVERGENiHBG005603.
InParanoidiQ14534.
KOiK00511.
OMAiQNSFYRS.
OrthoDBiEOG091G03XY.
PhylomeDBiQ14534.
TreeFamiTF331056.

Enzyme and pathway databases

UniPathwayiUPA00767; UER00752.
BioCyciMetaCyc:HS02595-MONOMER.
ZFISH:HS02595-MONOMER.
ReactomeiR-HSA-191273. Cholesterol biosynthesis.
R-HSA-2426168. Activation of gene expression by SREBF (SREBP).
SABIO-RKQ14534.

Miscellaneous databases

ChiTaRSiSQLE. human.
GeneWikiiSqualene_monooxygenase.
GenomeRNAii6713.
PROiQ14534.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000104549.
CleanExiHS_SQLE.
ExpressionAtlasiQ14534. baseline and differential.
GenevisibleiQ14534. HS.

Family and domain databases

Gene3Di3.50.50.60. 2 hits.
InterProiIPR023753. FAD/NAD-binding_dom.
IPR013698. Squalene_epoxidase.
[Graphical view]
PfamiPF08491. SE. 1 hit.
[Graphical view]
SUPFAMiSSF51905. SSF51905. 2 hits.
ProtoNetiSearch...

Entry informationi

Entry nameiERG1_HUMAN
AccessioniPrimary (citable) accession number: Q14534
Secondary accession number(s): Q9UEK6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 18, 2010
Last modified: November 30, 2016
This is version 145 of the entry and version 3 of the sequence. [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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 8
    Human chromosome 8: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. SIMILARITY comments
    Index of protein domains and families

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.