Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

A disintegrin and metalloproteinase with thrombospondin motifs 3

Gene

ADAMTS3

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

Functioni

Cleaves the propeptides of type II collagen prior to fibril assembly. Does not act on types I and III collagens.

Caution

Has sometimes been referred to as ADAMTS4.Curated

Cofactori

Zn2+By similarityNote: Binds 1 zinc ion per subunit.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi398Zinc; catalyticPROSITE-ProRule annotation1
Active sitei399PROSITE-ProRule annotation1
Metal bindingi402Zinc; catalyticPROSITE-ProRule annotation1
Metal bindingi408Zinc; catalyticPROSITE-ProRule annotation1

GO - Molecular functioni

  • endopeptidase activity Source: BHF-UCL
  • heparin binding Source: UniProtKB-KW
  • metalloendopeptidase activity Source: Reactome
  • zinc ion binding Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionHeparin-binding, Hydrolase, Metalloprotease, Protease
LigandMetal-binding, Zinc

Enzyme and pathway databases

ReactomeiR-HSA-1650814 Collagen biosynthesis and modifying enzymes
R-HSA-5083635 Defective B3GALTL causes Peters-plus syndrome (PpS)
R-HSA-5173214 O-glycosylation of TSR domain-containing proteins

Protein family/group databases

MEROPSiM12.220

Names & Taxonomyi

Protein namesi
Recommended name:
A disintegrin and metalloproteinase with thrombospondin motifs 3 (EC:3.4.24.-)
Short name:
ADAM-TS 3
Short name:
ADAM-TS3
Short name:
ADAMTS-3
Alternative name(s):
Procollagen II N-proteinase
Short name:
PC II-NP
Procollagen II amino propeptide-processing enzyme
Gene namesi
Name:ADAMTS3
Synonyms:KIAA0366
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000156140.8
HGNCiHGNC:219 ADAMTS3
MIMi605011 gene
neXtProtiNX_O15072

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

Extracellular matrix, Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi9508
MalaCardsiADAMTS3
OpenTargetsiENSG00000156140
Orphaneti2136 Hennekam syndrome
PharmGKBiPA24547

Polymorphism and mutation databases

BioMutaiADAMTS3

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 20Sequence analysisAdd BLAST20
PropeptideiPRO_000002916221 – 249By similarityAdd BLAST229
ChainiPRO_0000029163250 – 1205A disintegrin and metalloproteinase with thrombospondin motifs 3Add BLAST956

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi83N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi119N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi242N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi333 ↔ 382By similarity
Glycosylationi345N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi376 ↔ 455By similarity
Disulfide bondi415 ↔ 441By similarity
Glycosylationi475N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi482 ↔ 507By similarity
Disulfide bondi493 ↔ 516By similarity
Disulfide bondi502 ↔ 535By similarity
Disulfide bondi529 ↔ 540By similarity
Disulfide bondi563 ↔ 600By similarity
Disulfide bondi567 ↔ 605By similarity
Disulfide bondi578 ↔ 590By similarity
Glycosylationi814N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi942N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi978 ↔ 1010By similarity
Disulfide bondi982 ↔ 1015By similarity
Disulfide bondi993 ↔ 999By similarity

Post-translational modificationi

The precursor is cleaved by a furin endopeptidase.By similarity
Glycosylated. Can be O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X2-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS family members. Also can be C-glycosylated with one or two mannose molecules on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and N-glycosylated. These other glycosylations can also facilitate secretion (By similarity).By similarity

Keywords - PTMi

Cleavage on pair of basic residues, Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

EPDiO15072
PaxDbiO15072
PeptideAtlasiO15072
PRIDEiO15072
ProteomicsDBi48427

PTM databases

iPTMnetiO15072
PhosphoSitePlusiO15072

Expressioni

Tissue specificityi

Found in cartilage and skin.

Gene expression databases

BgeeiENSG00000156140 Expressed in 136 organ(s), highest expression level in caudate nucleus
CleanExiHS_ADAMTS3
GenevisibleiO15072 HS

Organism-specific databases

HPAiHPA021368
HPA021369

Interactioni

Protein-protein interaction databases

IntActiO15072, 1 interactor
STRINGi9606.ENSP00000286657

Structurei

3D structure databases

ProteinModelPortaliO15072
SMRiO15072
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini256 – 460Peptidase M12BPROSITE-ProRule annotationAdd BLAST205
Domaini470 – 550DisintegrinAdd BLAST81
Domaini551 – 606TSP type-1 1PROSITE-ProRule annotationAdd BLAST56
Domaini845 – 905TSP type-1 2PROSITE-ProRule annotationAdd BLAST61
Domaini906 – 965TSP type-1 3PROSITE-ProRule annotationAdd BLAST60
Domaini966 – 1014TSP type-1 4PROSITE-ProRule annotationAdd BLAST49
Domaini1015 – 1054PLACPROSITE-ProRule annotationAdd BLAST40

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni713 – 844SpacerAdd BLAST132

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi246 – 249Poly-Arg4
Compositional biasi608 – 712Cys-richAdd BLAST105

Domaini

The spacer domain and the TSP type-1 domains are important for a tight interaction with the extracellular matrix.

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

eggNOGiENOG410INDA Eukaryota
ENOG410XSRH LUCA
GeneTreeiENSGT00900000140815
HOGENOMiHOG000034222
HOVERGENiHBG004314
InParanoidiO15072
KOiK08619
OMAiPYEHPDS
OrthoDBiEOG091G0790
PhylomeDBiO15072
TreeFamiTF313537

Family and domain databases

Gene3Di2.20.100.10, 4 hits
3.40.390.10, 1 hit
InterProiView protein in InterPro
IPR010294 ADAM_spacer1
IPR013273 ADAMTS/ADAMTS-like
IPR024079 MetalloPept_cat_dom_sf
IPR001590 Peptidase_M12B
IPR002870 Peptidase_M12B_N
IPR010909 PLAC
IPR000884 TSP1_rpt
IPR036383 TSP1_rpt_sf
PfamiView protein in Pfam
PF05986 ADAM_spacer1, 1 hit
PF01562 Pep_M12B_propep, 1 hit
PF01421 Reprolysin, 1 hit
PF00090 TSP_1, 4 hits
PRINTSiPR01857 ADAMTSFAMILY
SMARTiView protein in SMART
SM00209 TSP1, 4 hits
SUPFAMiSSF82895 SSF82895, 4 hits
PROSITEiView protein in PROSITE
PS50215 ADAM_MEPRO, 1 hit
PS50900 PLAC, 1 hit
PS50092 TSP1, 4 hits

Sequencei

Sequence statusi: Complete.

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

O15072-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MVLLSLWLIA AALVEVRTSA DGQAGNEEMV QIDLPIKRYR EYELVTPVST
60 70 80 90 100
NLEGRYLSHT LSASHKKRSA RDVSSNPEQL FFNITAFGKD FHLRLKPNTQ
110 120 130 140 150
LVAPGAVVEW HETSLVPGNI TDPINNHQPG SATYRIRRTE PLQTNCAYVG
160 170 180 190 200
DIVDIPGTSV AISNCDGLAG MIKSDNEEYF IEPLERGKQM EEEKGRIHVV
210 220 230 240 250
YKRSAVEQAP IDMSKDFHYR ESDLEGLDDL GTVYGNIHQQ LNETMRRRRH
260 270 280 290 300
AGENDYNIEV LLGVDDSVVR FHGKEHVQNY LLTLMNIVNE IYHDESLGVH
310 320 330 340 350
INVVLVRMIM LGYAKSISLI ERGNPSRSLE NVCRWASQQQ RSDLNHSEHH
360 370 380 390 400
DHAIFLTRQD FGPAGMQGYA PVTGMCHPVR SCTLNHEDGF SSAFVVAHET
410 420 430 440 450
GHVLGMEHDG QGNRCGDETA MGSVMAPLVQ AAFHRYHWSR CSGQELKRYI
460 470 480 490 500
HSYDCLLDDP FDHDWPKLPE LPGINYSMDE QCRFDFGVGY KMCTAFRTFD
510 520 530 540 550
PCKQLWCSHP DNPYFCKTKK GPPLDGTECA AGKWCYKGHC MWKNANQQKQ
560 570 580 590 600
DGNWGSWTKF GSCSRTCGTG VRFRTRQCNN PMPINGGQDC PGVNFEYQLC
610 620 630 640 650
NTEECQKHFE DFRAQQCQQR NSHFEYQNTK HHWLPYEHPD PKKRCHLYCQ
660 670 680 690 700
SKETGDVAYM KQLVHDGTHC SYKDPYSICV RGECVKVGCD KEIGSNKVED
710 720 730 740 750
KCGVCGGDNS HCRTVKGTFT RTPRKLGYLK MFDIPPGARH VLIQEDEASP
760 770 780 790 800
HILAIKNQAT GHYILNGKGE EAKSRTFIDL GVEWDYNIED DIESLHTDGP
810 820 830 840 850
LHDPVIVLII PQENDTRSSL TYKYIIHEDS VPTINSNNVI QEELDTFEWA
860 870 880 890 900
LKSWSQCSKP CGGGFQYTKY GCRRKSDNKM VHRSFCEANK KPKPIRRMCN
910 920 930 940 950
IQECTHPLWV AEEWEHCTKT CGSSGYQLRT VRCLQPLLDG TNRSVHSKYC
960 970 980 990 1000
MGDRPESRRP CNRVPCPAQW KTGPWSECSV TCGEGTEVRQ VLCRAGDHCD
1010 1020 1030 1040 1050
GEKPESVRAC QLPPCNDEPC LGDKSIFCQM EVLARYCSIP GYNKLCCESC
1060 1070 1080 1090 1100
SKRSSTLPPP YLLEAAETHD DVISNPSDLP RSLVMPTSLV PYHSETPAKK
1110 1120 1130 1140 1150
MSLSSISSVG GPNAYAAFRP NSKPDGANLR QRSAQQAGSK TVRLVTVPSS
1160 1170 1180 1190 1200
PPTKRVHLSS ASQMAAASFF AASDSIGASS QARTSKKDGK IIDNRRPTRS

STLER
Length:1,205
Mass (Da):135,603
Last modified:January 11, 2011 - v4
Checksum:i01FA661B1C6BFF1B
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti857C → V in BAA20821 (PubMed:9205841).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_055012138R → K3 PublicationsCorresponds to variant dbSNP:rs788908Ensembl.1
Natural variantiVAR_0550131074S → P. Corresponds to variant dbSNP:rs35864003Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC093790 Genomic DNA No translation available.
AC095056 Genomic DNA No translation available.
AC104814 Genomic DNA No translation available.
BC130287 mRNA Translation: AAI30288.1
BC132735 mRNA Translation: AAI32736.1
AF247668 mRNA Translation: AAK28400.1
AB002364 mRNA Translation: BAA20821.1
CCDSiCCDS3553.1
RefSeqiNP_055058.2, NM_014243.2
UniGeneiHs.590919

Genome annotation databases

EnsembliENST00000286657; ENSP00000286657; ENSG00000156140
ENST00000622135; ENSP00000480055; ENSG00000156140
GeneIDi9508
KEGGihsa:9508
UCSCiuc003hgk.2 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC093790 Genomic DNA No translation available.
AC095056 Genomic DNA No translation available.
AC104814 Genomic DNA No translation available.
BC130287 mRNA Translation: AAI30288.1
BC132735 mRNA Translation: AAI32736.1
AF247668 mRNA Translation: AAK28400.1
AB002364 mRNA Translation: BAA20821.1
CCDSiCCDS3553.1
RefSeqiNP_055058.2, NM_014243.2
UniGeneiHs.590919

3D structure databases

ProteinModelPortaliO15072
SMRiO15072
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiO15072, 1 interactor
STRINGi9606.ENSP00000286657

Protein family/group databases

MEROPSiM12.220

PTM databases

iPTMnetiO15072
PhosphoSitePlusiO15072

Polymorphism and mutation databases

BioMutaiADAMTS3

Proteomic databases

EPDiO15072
PaxDbiO15072
PeptideAtlasiO15072
PRIDEiO15072
ProteomicsDBi48427

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000286657; ENSP00000286657; ENSG00000156140
ENST00000622135; ENSP00000480055; ENSG00000156140
GeneIDi9508
KEGGihsa:9508
UCSCiuc003hgk.2 human

Organism-specific databases

CTDi9508
DisGeNETi9508
EuPathDBiHostDB:ENSG00000156140.8
GeneCardsiADAMTS3
H-InvDBiHIX0004274
HGNCiHGNC:219 ADAMTS3
HPAiHPA021368
HPA021369
MalaCardsiADAMTS3
MIMi605011 gene
neXtProtiNX_O15072
OpenTargetsiENSG00000156140
Orphaneti2136 Hennekam syndrome
PharmGKBiPA24547
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410INDA Eukaryota
ENOG410XSRH LUCA
GeneTreeiENSGT00900000140815
HOGENOMiHOG000034222
HOVERGENiHBG004314
InParanoidiO15072
KOiK08619
OMAiPYEHPDS
OrthoDBiEOG091G0790
PhylomeDBiO15072
TreeFamiTF313537

Enzyme and pathway databases

ReactomeiR-HSA-1650814 Collagen biosynthesis and modifying enzymes
R-HSA-5083635 Defective B3GALTL causes Peters-plus syndrome (PpS)
R-HSA-5173214 O-glycosylation of TSR domain-containing proteins

Miscellaneous databases

ChiTaRSiADAMTS3 human
GeneWikiiADAMTS3
GenomeRNAii9508
PROiPR:O15072
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000156140 Expressed in 136 organ(s), highest expression level in caudate nucleus
CleanExiHS_ADAMTS3
GenevisibleiO15072 HS

Family and domain databases

Gene3Di2.20.100.10, 4 hits
3.40.390.10, 1 hit
InterProiView protein in InterPro
IPR010294 ADAM_spacer1
IPR013273 ADAMTS/ADAMTS-like
IPR024079 MetalloPept_cat_dom_sf
IPR001590 Peptidase_M12B
IPR002870 Peptidase_M12B_N
IPR010909 PLAC
IPR000884 TSP1_rpt
IPR036383 TSP1_rpt_sf
PfamiView protein in Pfam
PF05986 ADAM_spacer1, 1 hit
PF01562 Pep_M12B_propep, 1 hit
PF01421 Reprolysin, 1 hit
PF00090 TSP_1, 4 hits
PRINTSiPR01857 ADAMTSFAMILY
SMARTiView protein in SMART
SM00209 TSP1, 4 hits
SUPFAMiSSF82895 SSF82895, 4 hits
PROSITEiView protein in PROSITE
PS50215 ADAM_MEPRO, 1 hit
PS50900 PLAC, 1 hit
PS50092 TSP1, 4 hits
ProtoNetiSearch...

Entry informationi

Entry nameiATS3_HUMAN
AccessioniPrimary (citable) accession number: O15072
Secondary accession number(s): A1L3U9, Q9BXZ8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 1, 2000
Last sequence update: January 11, 2011
Last modified: November 7, 2018
This is version 178 of the entry and version 4 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 4
    Human chromosome 4: 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again