UniProtKB - O95450 (ATS2_HUMAN)
A disintegrin and metalloproteinase with thrombospondin motifs 2
ADAMTS2
Functioni
Caution
Catalytic activityi
- Cleaves the N-propeptide of collagen chain alpha-1(I) at Pro-|-Gln and of alpha-1(II) and alpha-2(I) at Ala-|-Gln. EC:3.4.24.14
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 408 | Zinc; catalyticPROSITE-ProRule annotation | 1 | |
Active sitei | 409 | PROSITE-ProRule annotation | 1 | |
Metal bindingi | 412 | Zinc; catalyticPROSITE-ProRule annotation | 1 | |
Metal bindingi | 418 | Zinc; catalyticPROSITE-ProRule annotation | 1 |
GO - Molecular functioni
- metalloendopeptidase activity Source: GO_Central
- metallopeptidase activity Source: ProtInc
- zinc ion binding Source: InterPro
GO - Biological processi
- collagen catabolic process Source: UniProtKB-KW
- collagen fibril organization Source: Ensembl
- extracellular matrix organization Source: GO_Central
- lung development Source: Ensembl
- protein processing Source: Ensembl
- skin development Source: Ensembl
- spermatogenesis Source: Ensembl
Keywordsi
Molecular function | Hydrolase, Metalloprotease, Protease |
Biological process | Collagen degradation |
Ligand | Metal-binding, Zinc |
Enzyme and pathway databases
PathwayCommonsi | O95450 |
Reactomei | R-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
MEROPSi | M12.301 |
Names & Taxonomyi
Protein namesi | Recommended name: A disintegrin and metalloproteinase with thrombospondin motifs 2 (EC:3.4.24.14)Short name: ADAM-TS 2 Short name: ADAM-TS2 Short name: ADAMTS-2 Alternative name(s): Procollagen I N-proteinase Short name: PC I-NP Procollagen I/II amino propeptide-processing enzyme Procollagen N-endopeptidase Short name: pNPI |
Gene namesi | Name:ADAMTS2 Synonyms:PCINP, PCPNI |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:218, ADAMTS2 |
MIMi | 604539, gene |
neXtProti | NX_O95450 |
VEuPathDBi | HostDB:ENSG00000087116.13 |
Subcellular locationi
Extracellular region or secreted
- extracellular matrix By similarity
Extracellular region or secreted
- collagen-containing extracellular matrix Source: InterPro
- extracellular matrix Source: GO_Central
- extracellular region Source: Reactome
Keywords - Cellular componenti
Extracellular matrix, SecretedPathology & Biotechi
Involvement in diseasei
Ehlers-Danlos syndrome, dermatosparaxis type (EDSDERMS)1 Publication
Keywords - Diseasei
Ehlers-Danlos syndromeOrganism-specific databases
DisGeNETi | 9509 |
MalaCardsi | ADAMTS2 |
MIMi | 225410, phenotype |
OpenTargetsi | ENSG00000087116 |
Orphaneti | 1901, Dermatosparaxis Ehlers-Danlos syndrome |
PharmGKBi | PA24546 |
Miscellaneous databases
Pharosi | O95450, Tbio |
Genetic variation databases
BioMutai | ADAMTS2 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 29 | Sequence analysisAdd BLAST | 29 | |
PropeptideiPRO_0000029158 | 30 – 253 | By similarityAdd BLAST | 224 | |
ChainiPRO_0000029159 | 254 – 1211 | A disintegrin and metalloproteinase with thrombospondin motifs 2Add BLAST | 958 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 112 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 251 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Disulfide bondi | 343 ↔ 392 | By similarity | ||
Disulfide bondi | 386 ↔ 465 | By similarity | ||
Disulfide bondi | 425 ↔ 451 | By similarity | ||
Disulfide bondi | 492 ↔ 517 | By similarity | ||
Disulfide bondi | 503 ↔ 526 | By similarity | ||
Disulfide bondi | 512 ↔ 545 | By similarity | ||
Disulfide bondi | 539 ↔ 550 | By similarity | ||
Disulfide bondi | 573 ↔ 610 | By similarity | ||
Disulfide bondi | 577 ↔ 615 | By similarity | ||
Disulfide bondi | 588 ↔ 600 | By similarity | ||
Glycosylationi | 949 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Disulfide bondi | 987 ↔ 1023 | By similarity | ||
Disulfide bondi | 991 ↔ 1028 | By similarity | ||
Glycosylationi | 993 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Disulfide bondi | 1002 ↔ 1012 | By similarity | ||
Glycosylationi | 1031 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 1098 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 1145 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 1150 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Post-translational modificationi
Keywords - PTMi
Disulfide bond, Glycoprotein, ZymogenProteomic databases
jPOSTi | O95450 |
MassIVEi | O95450 |
PaxDbi | O95450 |
PeptideAtlasi | O95450 |
PRIDEi | O95450 |
ProteomicsDBi | 50883 [O95450-1] 50884 [O95450-2] |
PTM databases
GlyGeni | O95450, 9 sites, 1 O-linked glycan (1 site) |
iPTMneti | O95450 |
PhosphoSitePlusi | O95450 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000087116, Expressed in female gonad and 182 other tissues |
ExpressionAtlasi | O95450, baseline and differential |
Genevisiblei | O95450, HS |
Organism-specific databases
HPAi | ENSG00000087116, Low tissue specificity |
Interactioni
Subunit structurei
May belong to a multimeric complex. Binds specifically to collagen type XIV (By similarity).
By similarityProtein-protein interaction databases
BioGRIDi | 114887, 27 interactors |
IntActi | O95450, 16 interactors |
STRINGi | 9606.ENSP00000251582 |
Miscellaneous databases
RNActi | O95450, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 266 – 470 | Peptidase M12BPROSITE-ProRule annotationAdd BLAST | 205 | |
Domaini | 480 – 560 | DisintegrinAdd BLAST | 81 | |
Domaini | 561 – 616 | TSP type-1 1PROSITE-ProRule annotationAdd BLAST | 56 | |
Domaini | 854 – 912 | TSP type-1 2PROSITE-ProRule annotationAdd BLAST | 59 | |
Domaini | 914 – 971 | TSP type-1 3PROSITE-ProRule annotationAdd BLAST | 58 | |
Domaini | 975 – 1029 | TSP type-1 4PROSITE-ProRule annotationAdd BLAST | 55 | |
Domaini | 1059 – 1097 | PLACPROSITE-ProRule annotationAdd BLAST | 39 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 723 – 851 | SpacerAdd BLAST | 129 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 691 – 693 | Cell attachment siteSequence analysis | 3 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 40 – 43 | Poly-Ala | 4 | |
Compositional biasi | 185 – 188 | Poly-Glu | 4 | |
Compositional biasi | 618 – 722 | Cys-richAdd BLAST | 105 |
Domaini
Keywords - Domaini
Repeat, SignalPhylogenomic databases
eggNOGi | KOG3538, Eukaryota |
GeneTreei | ENSGT00940000156647 |
HOGENOMi | CLU_000660_4_1_1 |
InParanoidi | O95450 |
OMAi | SVGYEWA |
OrthoDBi | 79609at2759 |
PhylomeDBi | O95450 |
TreeFami | TF313537 |
Family and domain databases
Gene3Di | 2.20.100.10, 4 hits 3.40.390.10, 1 hit |
InterProi | View protein in InterPro IPR041645, ADAM_CR_2 IPR010294, ADAM_spacer1 IPR013273, ADAMTS/ADAMTS-like IPR024079, MetalloPept_cat_dom_sf IPR013275, Pept_M12B_ADAM-TS2 IPR001590, Peptidase_M12B IPR002870, Peptidase_M12B_N IPR010909, PLAC IPR000884, TSP1_rpt IPR036383, TSP1_rpt_sf |
Pfami | View protein in Pfam PF17771, ADAM_CR_2, 1 hit PF05986, ADAM_spacer1, 1 hit PF01562, Pep_M12B_propep, 1 hit PF01421, Reprolysin, 1 hit PF00090, TSP_1, 1 hit |
PRINTSi | PR01859, ADAMTS2 PR01857, ADAMTSFAMILY |
SMARTi | View protein in SMART SM00209, TSP1, 4 hits |
SUPFAMi | SSF82895, SSF82895, 4 hits |
PROSITEi | View protein in PROSITE PS50215, ADAM_MEPRO, 1 hit PS50900, PLAC, 1 hit PS50092, TSP1, 4 hits |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MDPPAGAARR LLCPALLLLL LLLPPPLLPP PPPPANARLA AAADPPGGPL
60 70 80 90 100
GHGAERILAV PVRTDAQGRL VSHVVSAATS RAGVRARRAA PVRTPSFPGG
110 120 130 140 150
NEEEPGSHLF YNVTVFGRDL HLRLRPNARL VAPGATMEWQ GEKGTTRVEP
160 170 180 190 200
LLGSCLYVGD VAGLAEASSV ALSNCDGLAG LIRMEEEEFF IEPLEKGLAA
210 220 230 240 250
QEAEQGRVHV VYRRPPTSPP LGGPQALDTG ASLDSLDSLS RALGVLEEHA
260 270 280 290 300
NSSRRRARRH AADDDYNIEV LLGVDDSVVQ FHGKEHVQKY LLTLMNIVNE
310 320 330 340 350
IYHDESLGAH INVVLVRIIL LSYGKSMSLI EIGNPSQSLE NVCRWAYLQQ
360 370 380 390 400
KPDTGHDEYH DHAIFLTRQD FGPSGMQGYA PVTGMCHPVR SCTLNHEDGF
410 420 430 440 450
SSAFVVAHET GHVLGMEHDG QGNRCGDEVR LGSIMAPLVQ AAFHRFHWSR
460 470 480 490 500
CSQQELSRYL HSYDCLLDDP FAHDWPALPQ LPGLHYSMNE QCRFDFGLGY
510 520 530 540 550
MMCTAFRTFD PCKQLWCSHP DNPYFCKTKK GPPLDGTMCA PGKHCFKGHC
560 570 580 590 600
IWLTPDILKR DGSWGAWSPF GSCSRTCGTG VKFRTRQCDN PHPANGGRTC
610 620 630 640 650
SGLAYDFQLC SRQDCPDSLA DFREEQCRQW DLYFEHGDAQ HHWLPHEHRD
660 670 680 690 700
AKERCHLYCE SRETGEVVSM KRMVHDGTRC SYKDAFSLCV RGDCRKVGCD
710 720 730 740 750
GVIGSSKQED KCGVCGGDNS HCKVVKGTFT RSPKKHGYIK MFEIPAGARH
760 770 780 790 800
LLIQEVDATS HHLAVKNLET GKFILNEEND VDASSKTFIA MGVEWEYRDE
810 820 830 840 850
DGRETLQTMG PLHGTITVLV IPVGDTRVSL TYKYMIHEDS LNVDDNNVLE
860 870 880 890 900
EDSVVYEWAL KKWSPCSKPC GGGSQFTKYG CRRRLDHKMV HRGFCAALSK
910 920 930 940 950
PKAIRRACNP QECSQPVWVT GEWEPCSQTC GRTGMQVRSV RCIQPLHDNT
960 970 980 990 1000
TRSVHAKHCN DARPESRRAC SRELCPGRWR AGPWSQCSVT CGNGTQERPV
1010 1020 1030 1040 1050
LCRTADDSFG ICQEERPETA RTCRLGPCPR NISDPSKKSY VVQWLSRPDP
1060 1070 1080 1090 1100
DSPIRKISSK GHCQGDKSIF CRMEVLSRYC SIPGYNKLCC KSCNLYNNLT
1110 1120 1130 1140 1150
NVEGRIEPPP GKHNDIDVFM PTLPVPTVAM EVRPSPSTPL EVPLNASSTN
1160 1170 1180 1190 1200
ATEDHPETNA VDEPYKIHGL EDEVQPPNLI PRRPSPYEKT RNQRIQELID
1210
EMRKKEMLGK F
The sequence of this isoform differs from the canonical sequence as follows:
544-566: HCFKGHCIWLTPDILKRDGSWGA → FRPGAVAHACYPSTLGGQGRWIA
567-1211: Missing.
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketA0A1B0GTY3 | A0A1B0GTY3_HUMAN | A disintegrin and metalloproteinase... | ADAMTS2 | 1,094 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 1001 | L → P in CAA05880 (PubMed:10417273).Curated | 1 | |
Sequence conflicti | 1089 | C → S in CAA05880 (PubMed:10417273).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_047927 | 74 | V → M. Corresponds to variant dbSNP:rs2271211EnsemblClinVar. | 1 | |
Natural variantiVAR_047928 | 241 | R → H. Corresponds to variant dbSNP:rs11750821EnsemblClinVar. | 1 | |
Natural variantiVAR_020058 | 245 | V → I. Corresponds to variant dbSNP:rs398829EnsemblClinVar. | 1 | |
Natural variantiVAR_047929 | 331 | E → K. Corresponds to variant dbSNP:rs17667857EnsemblClinVar. | 1 | |
Natural variantiVAR_047930 | 665 | G → R. Corresponds to variant dbSNP:rs35372714EnsemblClinVar. | 1 | |
Natural variantiVAR_047931 | 827 | R → Q. Corresponds to variant dbSNP:rs35445112EnsemblClinVar. | 1 | |
Natural variantiVAR_020059 | 1177 | P → S. Corresponds to variant dbSNP:rs1054480EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_005497 | 544 – 566 | HCFKG…GSWGA → FRPGAVAHACYPSTLGGQGR WIA in isoform SpNPI. 1 PublicationAdd BLAST | 23 | |
Alternative sequenceiVSP_005498 | 567 – 1211 | Missing in isoform SpNPI. 1 PublicationAdd BLAST | 645 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AJ003125 mRNA Translation: CAA05880.1 AC008544 Genomic DNA No translation available. AC010216 Genomic DNA No translation available. AC109479 Genomic DNA No translation available. |
CCDSi | CCDS34311.1 [O95450-2] CCDS4444.1 [O95450-1] |
RefSeqi | NP_055059.2, NM_014244.4 [O95450-1] NP_067610.1, NM_021599.3 [O95450-2] |
Genome annotation databases
Ensembli | ENST00000251582; ENSP00000251582; ENSG00000087116 [O95450-1] ENST00000274609; ENSP00000274609; ENSG00000087116 [O95450-2] ENST00000639107; ENSP00000492365; ENSG00000283802 [O95450-2] ENST00000639986; ENSP00000492346; ENSG00000283802 [O95450-1] |
GeneIDi | 9509 |
KEGGi | hsa:9509 |
UCSCi | uc003mjw.3, human [O95450-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AJ003125 mRNA Translation: CAA05880.1 AC008544 Genomic DNA No translation available. AC010216 Genomic DNA No translation available. AC109479 Genomic DNA No translation available. |
CCDSi | CCDS34311.1 [O95450-2] CCDS4444.1 [O95450-1] |
RefSeqi | NP_055059.2, NM_014244.4 [O95450-1] NP_067610.1, NM_021599.3 [O95450-2] |
3D structure databases
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Protein-protein interaction databases
BioGRIDi | 114887, 27 interactors |
IntActi | O95450, 16 interactors |
STRINGi | 9606.ENSP00000251582 |
Protein family/group databases
MEROPSi | M12.301 |
PTM databases
GlyGeni | O95450, 9 sites, 1 O-linked glycan (1 site) |
iPTMneti | O95450 |
PhosphoSitePlusi | O95450 |
Genetic variation databases
BioMutai | ADAMTS2 |
Proteomic databases
jPOSTi | O95450 |
MassIVEi | O95450 |
PaxDbi | O95450 |
PeptideAtlasi | O95450 |
PRIDEi | O95450 |
ProteomicsDBi | 50883 [O95450-1] 50884 [O95450-2] |
Protocols and materials databases
Antibodypediai | 17642, 176 antibodies |
DNASUi | 9509 |
Genome annotation databases
Ensembli | ENST00000251582; ENSP00000251582; ENSG00000087116 [O95450-1] ENST00000274609; ENSP00000274609; ENSG00000087116 [O95450-2] ENST00000639107; ENSP00000492365; ENSG00000283802 [O95450-2] ENST00000639986; ENSP00000492346; ENSG00000283802 [O95450-1] |
GeneIDi | 9509 |
KEGGi | hsa:9509 |
UCSCi | uc003mjw.3, human [O95450-1] |
Organism-specific databases
CTDi | 9509 |
DisGeNETi | 9509 |
GeneCardsi | ADAMTS2 |
HGNCi | HGNC:218, ADAMTS2 |
HPAi | ENSG00000087116, Low tissue specificity |
MalaCardsi | ADAMTS2 |
MIMi | 225410, phenotype 604539, gene |
neXtProti | NX_O95450 |
OpenTargetsi | ENSG00000087116 |
Orphaneti | 1901, Dermatosparaxis Ehlers-Danlos syndrome |
PharmGKBi | PA24546 |
VEuPathDBi | HostDB:ENSG00000087116.13 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG3538, Eukaryota |
GeneTreei | ENSGT00940000156647 |
HOGENOMi | CLU_000660_4_1_1 |
InParanoidi | O95450 |
OMAi | SVGYEWA |
OrthoDBi | 79609at2759 |
PhylomeDBi | O95450 |
TreeFami | TF313537 |
Enzyme and pathway databases
PathwayCommonsi | O95450 |
Reactomei | R-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
BioGRID-ORCSi | 9509, 9 hits in 873 CRISPR screens |
ChiTaRSi | ADAMTS2, human |
GeneWikii | ADAMTS2 |
GenomeRNAii | 9509 |
Pharosi | O95450, Tbio |
PROi | PR:O95450 |
RNActi | O95450, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000087116, Expressed in female gonad and 182 other tissues |
ExpressionAtlasi | O95450, baseline and differential |
Genevisiblei | O95450, HS |
Family and domain databases
Gene3Di | 2.20.100.10, 4 hits 3.40.390.10, 1 hit |
InterProi | View protein in InterPro IPR041645, ADAM_CR_2 IPR010294, ADAM_spacer1 IPR013273, ADAMTS/ADAMTS-like IPR024079, MetalloPept_cat_dom_sf IPR013275, Pept_M12B_ADAM-TS2 IPR001590, Peptidase_M12B IPR002870, Peptidase_M12B_N IPR010909, PLAC IPR000884, TSP1_rpt IPR036383, TSP1_rpt_sf |
Pfami | View protein in Pfam PF17771, ADAM_CR_2, 1 hit PF05986, ADAM_spacer1, 1 hit PF01562, Pep_M12B_propep, 1 hit PF01421, Reprolysin, 1 hit PF00090, TSP_1, 1 hit |
PRINTSi | PR01859, ADAMTS2 PR01857, ADAMTSFAMILY |
SMARTi | View protein in SMART SM00209, TSP1, 4 hits |
SUPFAMi | SSF82895, SSF82895, 4 hits |
PROSITEi | View protein in PROSITE PS50215, ADAM_MEPRO, 1 hit PS50900, PLAC, 1 hit PS50092, TSP1, 4 hits |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | ATS2_HUMAN | |
Accessioni | O95450Primary (citable) accession number: O95450 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | December 1, 2000 |
Last sequence update: | December 16, 2008 | |
Last modified: | February 10, 2021 | |
This is version 206 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Reference proteomeDocuments
- Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - Human chromosome 5
Human chromosome 5: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants