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Protein

Protein-lysine 6-oxidase

Gene

LOX

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

Functioni

Responsible for the post-translational oxidative deamination of peptidyl lysine residues in precursors to fibrous collagen and elastin (PubMed:26838787). Regulator of Ras expression. May play a role in tumor suppression. Plays a role in the aortic wall architecture (By similarity).By similarity1 Publication

Miscellaneous

The propeptide plays a role in directing the deposition of this enzyme to elastic fibers, via interaction with tropoelastin.By similarity

Catalytic activityi

[Protein]-L-lysine + O2 + H2O = [protein]-(S)-2-amino-6-oxohexanoate + NH3 + H2O2.1 Publication

Cofactori

Protein has several cofactor binding sites:

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi292CopperSequence analysis1
Metal bindingi294CopperSequence analysis1
Metal bindingi296CopperSequence analysis1

GO - Molecular functioni

  • copper ion binding Source: ProtInc
  • protein-lysine 6-oxidase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandCopper, Metal-binding

Enzyme and pathway databases

BRENDAi1.4.3.13 2681
ReactomeiR-HSA-1566948 Elastic fibre formation
R-HSA-2243919 Crosslinking of collagen fibrils
SIGNORiP28300

Names & Taxonomyi

Protein namesi
Recommended name:
Protein-lysine 6-oxidase (EC:1.4.3.131 Publication)
Alternative name(s):
Lysyl oxidase
Gene namesi
Name:LOX
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

EuPathDBiHostDB:ENSG00000113083.12
HGNCiHGNC:6664 LOX
MIMi153455 gene
neXtProtiNX_P28300

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

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Aortic aneurysm, familial thoracic 10 (AAT10)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of thoracic aortic aneurysm, a disease characterized by permanent dilation of the thoracic aorta usually due to degenerative changes in the aortic wall. It is primarily associated with a characteristic histologic appearance known as 'medial necrosis' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance.
See also OMIM:617168
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07753479A → T in AAT10; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs752839330EnsemblClinVar.1
Natural variantiVAR_077535154L → F in AAT10; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs767855588Ensembl.1
Natural variantiVAR_077536248T → I in AAT10; unknown pathological significance; 8% decrease of lysyl oxidase activity. 1 Publication1
Natural variantiVAR_077537267Q → P in AAT10. 1 PublicationCorresponds to variant dbSNP:rs886040967EnsemblClinVar.1
Natural variantiVAR_077538280S → I in AAT10; 50% decrease of lysyl oxidase activity. 1 PublicationCorresponds to variant dbSNP:rs886040965EnsemblClinVar.1
Natural variantiVAR_077539298M → R in AAT10. 1 PublicationCorresponds to variant dbSNP:rs876657852EnsemblClinVar.1
Natural variantiVAR_077540348S → R in AAT10; 21% decrease of lysyl oxidase activity. 1 Publication1

Keywords - Diseasei

Aortic aneurysm, Disease mutation

Organism-specific databases

DisGeNETi4015
MalaCardsiLOX
MIMi617168 phenotype
OpenTargetsiENSG00000113083
Orphaneti91387 Familial thoracic aortic aneurysm and aortic dissection
PharmGKBiPA30427

Chemistry databases

ChEMBLiCHEMBL2249

Polymorphism and mutation databases

BioMutaiLOX

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 21By similarityAdd BLAST21
PropeptideiPRO_000001852022 – 168By similarityAdd BLAST147
ChainiPRO_0000018521169 – 417Protein-lysine 6-oxidaseAdd BLAST249

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi81N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi97N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi144N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi238 ↔ 244By similarity
Disulfide bondi291 ↔ 340By similarity
Cross-linki320 ↔ 355Lysine tyrosylquinone (Lys-Tyr)By similarity
Disulfide bondi324 ↔ 330By similarity
Disulfide bondi351 ↔ 361By similarity
Modified residuei3552',4',5'-topaquinoneBy similarity1
Disulfide bondi398 ↔ 412By similarity

Post-translational modificationi

The lysine tyrosylquinone cross-link (LTQ) is generated by condensation of the epsilon-amino group of a lysine with a topaquinone produced by oxidation of tyrosine.By similarity
Proteolytically activated by BMP1.By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein, LTQ, TPQ

Proteomic databases

EPDiP28300
MaxQBiP28300
PaxDbiP28300
PeptideAtlasiP28300
PRIDEiP28300
ProteomicsDBi54461

PTM databases

GlyConnecti816
iPTMnetiP28300
PhosphoSitePlusiP28300
UniCarbKBiP28300

Miscellaneous databases

PMAP-CutDBiP28300

Expressioni

Tissue specificityi

Heart, placenta, skeletal muscle, kidney, lung and pancreas.1 Publication

Gene expression databases

BgeeiENSG00000113083 Expressed in 177 organ(s), highest expression level in smooth muscle tissue
CleanExiHS_LOX
ExpressionAtlasiP28300 baseline and differential
GenevisibleiP28300 HS

Organism-specific databases

HPAiHPA043930

Interactioni

Subunit structurei

Interacts with MFAP4 (PubMed:26601954).1 Publication

Binary interactionsi

Protein-protein interaction databases

BioGridi110199, 20 interactors
DIPiDIP-49007N
IntActiP28300, 43 interactors
MINTiP28300
STRINGi9606.ENSP00000231004

Chemistry databases

BindingDBiP28300

Structurei

3D structure databases

ProteinModelPortaliP28300
SMRiP28300
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni213 – 417Lysyl-oxidase likeAdd BLAST205

Sequence similaritiesi

Belongs to the lysyl oxidase family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IHTH Eukaryota
ENOG41101E4 LUCA
GeneTreeiENSGT00900000140830
HOGENOMiHOG000234262
HOVERGENiHBG000226
InParanoidiP28300
KOiK00277
OMAiLQWSHNG
OrthoDBiEOG091G02XD
PhylomeDBiP28300
TreeFamiTF326061

Family and domain databases

InterProiView protein in InterPro
IPR001695 Lysyl_oxidase
IPR019828 Lysyl_oxidase_CS
PfamiView protein in Pfam
PF01186 Lysyl_oxidase, 1 hit
PRINTSiPR00074 LYSYLOXIDASE
PROSITEiView protein in PROSITE
PS00926 LYSYL_OXIDASE, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P28300-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MRFAWTVLLL GPLQLCALVH CAPPAAGQQQ PPREPPAAPG AWRQQIQWEN
60 70 80 90 100
NGQVFSLLSL GSQYQPQRRR DPGAAVPGAA NASAQQPRTP ILLIRDNRTA
110 120 130 140 150
AARTRTAGSS GVTAGRPRPT ARHWFQAGYS TSRAREAGAS RAENQTAPGE
160 170 180 190 200
VPALSNLRPP SRVDGMVGDD PYNPYKYSDD NPYYNYYDTY ERPRPGGRYR
210 220 230 240 250
PGYGTGYFQY GLPDLVADPY YIQASTYVQK MSMYNLRCAA EENCLASTAY
260 270 280 290 300
RADVRDYDHR VLLRFPQRVK NQGTSDFLPS RPRYSWEWHS CHQHYHSMDE
310 320 330 340 350
FSHYDLLDAN TQRRVAEGHK ASFCLEDTSC DYGYHRRFAC TAHTQGLSPG
360 370 380 390 400
CYDTYGADID CQWIDITDVK PGNYILKVSV NPSYLVPESD YTNNVVRCDI
410
RYTGHHAYAS GCTISPY
Length:417
Mass (Da):46,944
Last modified:October 1, 1993 - v2
Checksum:i6412A78443E03F04
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0YAL3H0YAL3_HUMAN
Protein-lysine 6-oxidase
LOX
61Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti30Q → H (PubMed:7706256).Curated1
Sequence conflicti31P → A (PubMed:7706256).Curated1
Sequence conflicti95R → A (PubMed:7706256).Curated1
Sequence conflicti102A → G in AAB21243 (PubMed:1685472).Curated1
Sequence conflicti137A → R in AAA59525 (PubMed:1357535).Curated1
Sequence conflicti137A → R in AAB23549 (PubMed:1357535).Curated1
Sequence conflicti139A → P in AAB21243 (PubMed:1685472).Curated1
Sequence conflicti304 – 305YD → LY in AAB21243 (PubMed:1685472).Curated2
Sequence conflicti315V → W in AAB21243 (PubMed:1685472).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07753479A → T in AAT10; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs752839330EnsemblClinVar.1
Natural variantiVAR_077535154L → F in AAT10; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs767855588Ensembl.1
Natural variantiVAR_004282158R → Q2 PublicationsCorresponds to variant dbSNP:rs1800449EnsemblClinVar.1
Natural variantiVAR_077536248T → I in AAT10; unknown pathological significance; 8% decrease of lysyl oxidase activity. 1 Publication1
Natural variantiVAR_077537267Q → P in AAT10. 1 PublicationCorresponds to variant dbSNP:rs886040967EnsemblClinVar.1
Natural variantiVAR_077538280S → I in AAT10; 50% decrease of lysyl oxidase activity. 1 PublicationCorresponds to variant dbSNP:rs886040965EnsemblClinVar.1
Natural variantiVAR_077539298M → R in AAT10. 1 PublicationCorresponds to variant dbSNP:rs876657852EnsemblClinVar.1
Natural variantiVAR_077540348S → R in AAT10; 21% decrease of lysyl oxidase activity. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S78694 mRNA Translation: AAB21243.1
M94054 mRNA Translation: AAA59525.1
S45875 mRNA Translation: AAB23549.1
AF039291 mRNA Translation: AAD02130.1
AK312269 mRNA Translation: BAG35200.1
BC074820 mRNA Translation: AAH74820.1
BC074872 mRNA Translation: AAH74872.1
BC089436 mRNA Translation: AAH89436.1
L16895 Genomic DNA Translation: AAA16870.1
M84150 Genomic DNA Translation: AAA59541.1
CCDSiCCDS4129.1
PIRiA47529 OXHUL
RefSeqiNP_002308.2, NM_002317.6
UniGeneiHs.102267

Genome annotation databases

EnsembliENST00000231004; ENSP00000231004; ENSG00000113083
ENST00000639739; ENSP00000492324; ENSG00000113083
GeneIDi4015
KEGGihsa:4015
UCSCiuc003ksu.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S78694 mRNA Translation: AAB21243.1
M94054 mRNA Translation: AAA59525.1
S45875 mRNA Translation: AAB23549.1
AF039291 mRNA Translation: AAD02130.1
AK312269 mRNA Translation: BAG35200.1
BC074820 mRNA Translation: AAH74820.1
BC074872 mRNA Translation: AAH74872.1
BC089436 mRNA Translation: AAH89436.1
L16895 Genomic DNA Translation: AAA16870.1
M84150 Genomic DNA Translation: AAA59541.1
CCDSiCCDS4129.1
PIRiA47529 OXHUL
RefSeqiNP_002308.2, NM_002317.6
UniGeneiHs.102267

3D structure databases

ProteinModelPortaliP28300
SMRiP28300
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110199, 20 interactors
DIPiDIP-49007N
IntActiP28300, 43 interactors
MINTiP28300
STRINGi9606.ENSP00000231004

Chemistry databases

BindingDBiP28300
ChEMBLiCHEMBL2249

PTM databases

GlyConnecti816
iPTMnetiP28300
PhosphoSitePlusiP28300
UniCarbKBiP28300

Polymorphism and mutation databases

BioMutaiLOX

Proteomic databases

EPDiP28300
MaxQBiP28300
PaxDbiP28300
PeptideAtlasiP28300
PRIDEiP28300
ProteomicsDBi54461

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000231004; ENSP00000231004; ENSG00000113083
ENST00000639739; ENSP00000492324; ENSG00000113083
GeneIDi4015
KEGGihsa:4015
UCSCiuc003ksu.4 human

Organism-specific databases

CTDi4015
DisGeNETi4015
EuPathDBiHostDB:ENSG00000113083.12
GeneCardsiLOX
HGNCiHGNC:6664 LOX
HPAiHPA043930
MalaCardsiLOX
MIMi153455 gene
617168 phenotype
neXtProtiNX_P28300
OpenTargetsiENSG00000113083
Orphaneti91387 Familial thoracic aortic aneurysm and aortic dissection
PharmGKBiPA30427
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IHTH Eukaryota
ENOG41101E4 LUCA
GeneTreeiENSGT00900000140830
HOGENOMiHOG000234262
HOVERGENiHBG000226
InParanoidiP28300
KOiK00277
OMAiLQWSHNG
OrthoDBiEOG091G02XD
PhylomeDBiP28300
TreeFamiTF326061

Enzyme and pathway databases

BRENDAi1.4.3.13 2681
ReactomeiR-HSA-1566948 Elastic fibre formation
R-HSA-2243919 Crosslinking of collagen fibrils
SIGNORiP28300

Miscellaneous databases

ChiTaRSiLOX human
GeneWikiiLysyl_oxidase
GenomeRNAii4015
PMAP-CutDBiP28300
PROiPR:P28300
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000113083 Expressed in 177 organ(s), highest expression level in smooth muscle tissue
CleanExiHS_LOX
ExpressionAtlasiP28300 baseline and differential
GenevisibleiP28300 HS

Family and domain databases

InterProiView protein in InterPro
IPR001695 Lysyl_oxidase
IPR019828 Lysyl_oxidase_CS
PfamiView protein in Pfam
PF01186 Lysyl_oxidase, 1 hit
PRINTSiPR00074 LYSYLOXIDASE
PROSITEiView protein in PROSITE
PS00926 LYSYL_OXIDASE, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiLYOX_HUMAN
AccessioniPrimary (citable) accession number: P28300
Secondary accession number(s): B2R5Q3, Q5FWF0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: October 1, 1993
Last modified: November 7, 2018
This is version 183 of the entry and version 2 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. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
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