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Protein

Squalene monooxygenase

Gene

SQLE

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandFAD, Flavoprotein

Enzyme and pathway databases

BioCyciMetaCyc:HS02595-MONOMER
ReactomeiR-HSA-191273 Cholesterol biosynthesis
R-HSA-2426168 Activation of gene expression by SREBF (SREBP)
SABIO-RKiQ14534
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

EuPathDBiHostDB:ENSG00000104549.11
HGNCiHGNC:11279 SQLE
MIMi602019 gene
neXtProtiNX_Q14534

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
Transmembranei20 – 40HelicalSequence analysisAdd BLAST21
Transmembranei61 – 81HelicalSequence analysisAdd BLAST21
Transmembranei123 – 143HelicalSequence analysisAdd BLAST21
Transmembranei546 – 566HelicalSequence analysisAdd BLAST21

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane, Microsome

Pathology & Biotechi

Organism-specific databases

DisGeNETi6713
OpenTargetsiENSG00000104549
PharmGKBiPA36108

Chemistry databases

ChEMBLiCHEMBL3592
DrugBankiDB01091 Butenafine
DB08846 Ellagic Acid
DB00735 Naftifine
DB04927 NM100060
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
ProteomicsDBi60034

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, 16 interactors
IntActiQ14534, 4 interactors
MINTiQ14534
STRINGi9606.ENSP00000265896

Chemistry databases

BindingDBiQ14534

Structurei

3D structure databases

ProteinModelPortaliQ14534
SMRiQ14534
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
OMAiSSHFVGC
OrthoDBiEOG091G03XY
PhylomeDBiQ14534
TreeFamiTF331056

Family and domain databases

Gene3Di3.50.50.60, 3 hits
InterProiView protein in InterPro
IPR036188 FAD/NAD-bd_sf
IPR013698 Squalene_epoxidase
PfamiView protein in Pfam
PF08491 SE, 1 hit
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

Similar proteinsi

Entry informationi

Entry nameiERG1_HUMAN
AccessioniPrimary (citable) accession number: Q14534
Secondary accession number(s): Q9UEK6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 18, 2010
Last modified: June 20, 2018
This is version 157 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

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

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