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Protein

A disintegrin and metalloproteinase with thrombospondin motifs 10

Gene

ADAMTS10

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

Functioni

Metalloprotease that participate in microfibrils assembly. Microfibrils are extracellular matrix components occurring independently or along with elastin in the formation of elastic tissues.1 Publication

Cofactori

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi392Zinc; catalyticBy similarity1
Active sitei393PROSITE-ProRule annotation1
Metal bindingi396Zinc; catalyticBy similarity1
Metal bindingi402Zinc; catalyticBy similarity1

GO - Molecular functioni

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Metal-binding, Zinc

Enzyme and pathway databases

BioCyciZFISH:ENSG00000142303-MONOMER.
ReactomeiR-HSA-5083635. Defective B3GALTL causes Peters-plus syndrome (PpS).
R-HSA-5173214. O-glycosylation of TSR domain-containing proteins.

Protein family/group databases

MEROPSiM12.235.

Names & Taxonomyi

Protein namesi
Recommended name:
A disintegrin and metalloproteinase with thrombospondin motifs 10 (EC:3.4.24.-)
Short name:
ADAM-TS 10
Short name:
ADAM-TS10
Short name:
ADAMTS-10
Gene namesi
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:13201. ADAMTS10.

Subcellular locationi

GO - Cellular componenti

  • extracellular matrix Source: UniProtKB
  • microfibril Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Extracellular matrix, Secreted

Pathology & Biotechi

Involvement in diseasei

Weill-Marchesani syndrome 1 (WMS1)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma.
See also OMIM:277600
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05443925A → T in WMS1; shows consistent and significantly diminished protein secretion. 1 Publication1

Keywords - Diseasei

Disease mutation, Dwarfism

Organism-specific databases

DisGeNETi81794.
MalaCardsiADAMTS10.
MIMi277600. phenotype.
OpenTargetsiENSG00000142303.
Orphaneti3449. Weill-Marchesani syndrome.
PharmGKBiPA24537.

Polymorphism and mutation databases

DMDMi148887344.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 25Sequence analysisAdd BLAST25
PropeptideiPRO_000002918426 – 233By similarityAdd BLAST208
ChainiPRO_0000029185234 – 1103A disintegrin and metalloproteinase with thrombospondin motifs 10Add BLAST870

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi90N-linked (GlcNAc...)Sequence analysis1
Glycosylationi222N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi315 ↔ 376By similarity
Glycosylationi323N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi351 ↔ 358By similarity
Disulfide bondi370 ↔ 452By similarity
Disulfide bondi409 ↔ 436By similarity
Disulfide bondi479 ↔ 501By similarity
Disulfide bondi490 ↔ 508By similarity
Disulfide bondi496 ↔ 531By similarity
Disulfide bondi521 ↔ 536By similarity
Disulfide bondi559 ↔ 596By similarity
Disulfide bondi563 ↔ 601By similarity
Disulfide bondi574 ↔ 586By similarity
Glycosylationi740N-linked (GlcNAc...)Sequence analysis1
Glycosylationi795N-linked (GlcNAc...)Sequence analysis1
Glycosylationi892N-linked (GlcNAc...)Sequence analysis1

Post-translational modificationi

Glycosylated. Can be O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(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

PaxDbiQ9H324.
PeptideAtlasiQ9H324.
PRIDEiQ9H324.

PTM databases

iPTMnetiQ9H324.
PhosphoSitePlusiQ9H324.

Expressioni

Tissue specificityi

Widely expressed in adult tissues.1 Publication

Gene expression databases

BgeeiENSG00000142303.
CleanExiHS_ADAMTS10.
ExpressionAtlasiQ9H324. baseline and differential.
GenevisibleiQ9H324. HS.

Organism-specific databases

HPAiHPA040223.

Interactioni

Subunit structurei

Interacts with FBN1; this interaction promotes microfibrils assembly.1 Publication

Protein-protein interaction databases

BioGridi123586. 7 interactors.
IntActiQ9H324. 7 interactors.
MINTiMINT-8247349.
STRINGi9606.ENSP00000270328.

Structurei

3D structure databases

ProteinModelPortaliQ9H324.
SMRiQ9H324.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini239 – 457Peptidase M12BPROSITE-ProRule annotationAdd BLAST219
Domaini460 – 546DisintegrinAdd BLAST87
Domaini547 – 602TSP type-1 1PROSITE-ProRule annotationAdd BLAST56
Domaini825 – 883TSP type-1 2PROSITE-ProRule annotationAdd BLAST59
Domaini884 – 945TSP type-1 3PROSITE-ProRule annotationAdd BLAST62
Domaini947 – 1001TSP type-1 4PROSITE-ProRule annotationAdd BLAST55
Domaini1003 – 1058TSP type-1 5PROSITE-ProRule annotationAdd BLAST56
Domaini1065 – 1103PLACPROSITE-ProRule annotationAdd BLAST39

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni706 – 828SpacerAdd BLAST123

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi58 – 61Poly-Pro4
Compositional biasi568 – 571Poly-Ser4
Compositional biasi604 – 705Cys-richAdd BLAST102

Domaini

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

Sequence similaritiesi

Contains 1 disintegrin domain.Curated
Contains 1 peptidase M12B domain.PROSITE-ProRule annotation
Contains 1 PLAC domain.PROSITE-ProRule annotation
Contains 5 TSP type-1 domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

eggNOGiKOG3538. Eukaryota.
ENOG410XPKZ. LUCA.
GeneTreeiENSGT00760000118880.
HOGENOMiHOG000004800.
HOVERGENiHBG004315.
InParanoidiQ9H324.
KOiK08625.
PhylomeDBiQ9H324.
TreeFamiTF313537.

Family and domain databases

Gene3Di3.40.390.10. 1 hit.
InterProiIPR010294. ADAM_spacer1.
IPR024079. MetalloPept_cat_dom.
IPR001590. Peptidase_M12B.
IPR013273. Peptidase_M12B_ADAM-TS.
IPR002870. Peptidase_M12B_N.
IPR010909. PLAC.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF05986. ADAM_spacer1. 1 hit.
PF01562. Pep_M12B_propep. 1 hit.
PF08686. PLAC. 1 hit.
PF01421. Reprolysin. 1 hit.
PF00090. TSP_1. 5 hits.
[Graphical view]
PRINTSiPR01857. ADAMTSFAMILY.
SMARTiSM00209. TSP1. 5 hits.
[Graphical view]
SUPFAMiSSF82895. SSF82895. 5 hits.
PROSITEiPS50215. ADAM_MEPRO. 1 hit.
PS50900. PLAC. 1 hit.
PS50092. TSP1. 5 hits.
PS00142. ZINC_PROTEASE. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9H324-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAPACQILRW ALALGLGLMF EVTHAFRSQD EFLSSLESYE IAFPTRVDHN
60 70 80 90 100
GALLAFSPPP PRRQRRGTGA TAESRLFYKV ASPSTHFLLN LTRSSRLLAG
110 120 130 140 150
HVSVEYWTRE GLAWQRAARP HCLYAGHLQG QASTSHVAIS TCGGLHGLIV
160 170 180 190 200
ADEEEYLIEP LHGGPKGSRS PEESGPHVVY KRSSLRHPHL DTACGVRDEK
210 220 230 240 250
PWKGRPWWLR TLKPPPARPL GNETERGQPG LKRSVSRERY VETLVVADKM
260 270 280 290 300
MVAYHGRRDV EQYVLAIMNI VAKLFQDSSL GSTVNILVTR LILLTEDQPT
310 320 330 340 350
LEITHHAGKS LDSFCKWQKS IVNHSGHGNA IPENGVANHD TAVLITRYDI
360 370 380 390 400
CIYKNKPCGT LGLAPVGGMC ERERSCSVNE DIGLATAFTI AHEIGHTFGM
410 420 430 440 450
NHDGVGNSCG ARGQDPAKLM AAHITMKTNP FVWSSCSRDY ITSFLDSGLG
460 470 480 490 500
LCLNNRPPRQ DFVYPTVAPG QAYDADEQCR FQHGVKSRQC KYGEVCSELW
510 520 530 540 550
CLSKSNRCIT NSIPAAEGTL CQTHTIDKGW CYKRVCVPFG SRPEGVDGAW
560 570 580 590 600
GPWTPWGDCS RTCGGGVSSS SRHCDSPRPT IGGKYCLGER RRHRSCNTDD
610 620 630 640 650
CPPGSQDFRE VQCSEFDSIP FRGKFYKWKT YRGGGVKACS LTCLAEGFNF
660 670 680 690 700
YTERAAAVVD GTPCRPDTVD ICVSGECKHV GCDRVLGSDL REDKCRVCGG
710 720 730 740 750
DGSACETIEG VFSPASPGAG YEDVVWIPKG SVHIFIQDLN LSLSHLALKG
760 770 780 790 800
DQESLLLEGL PGTPQPHRLP LAGTTFQLRQ GPDQVQSLEA LGPINASLIV
810 820 830 840 850
MVLARTELPA LRYRFNAPIA RDSLPPYSWH YAPWTKCSAQ CAGGSQVQAV
860 870 880 890 900
ECRNQLDSSA VAPHYCSAHS KLPKRQRACN TEPCPPDWVV GNWSLCSRSC
910 920 930 940 950
DAGVRSRSVV CQRRVSAAEE KALDDSACPQ PRPPVLEACH GPTCPPEWAA
960 970 980 990 1000
LDWSECTPSC GPGLRHRVVL CKSADHRATL PPAHCSPAAK PPATMRCNLR
1010 1020 1030 1040 1050
RCPPARWVAG EWGECSAQCG VGQRQRSVRC TSHTGQASHE CTEALRPPTT
1060 1070 1080 1090 1100
QQCEAKCDSP TPGDGPEECK DVNKVAYCPL VLKFQFCSRA YFRQMCCKTC

HGH
Length:1,103
Mass (Da):120,874
Last modified:June 12, 2007 - v2
Checksum:iD7558271C303A87C
GO
Isoform 2 (identifier: Q9H324-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-637: MAPACQILRW...WKTYRGGGVK → MGPTSVLRAG...NSTSGKRTGE

Note: No experimental confirmation available. Gene prediction based on partial mRNA and EST data.
Show »
Length:590
Mass (Da):62,530
Checksum:i346AE6537FB016C6
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti385 – 386AT → PQ in AAG35563 (PubMed:15355968).Curated2
Sequence conflicti437S → N in AAG35563 (PubMed:15355968).Curated1
Sequence conflicti643C → S in AAG35563 (PubMed:15355968).Curated1
Sequence conflicti1101H → Q in AAG35563 (PubMed:15355968).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05443925A → T in WMS1; shows consistent and significantly diminished protein secretion. 1 Publication1
Natural variantiVAR_054440119R → Q.Corresponds to variant rs3814291dbSNPEnsembl.1
Natural variantiVAR_054441134T → S.1 PublicationCorresponds to variant rs7255721dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0547071 – 637MAPAC…GGGVK → MGPTSVLRAGLTPSCLPPPS GATNGSVSPLGRAQRVWTEP GGRGLHGATAAGPVAAACPL LAVTATAPGQPSGASTVWVR EGGTAPATRMTVPLAPRTSE KCSVLNLTASLSVGNSTSGK RTGE in isoform 2. CuratedAdd BLAST637

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC092315 Genomic DNA. No translation available.
AC130469 Genomic DNA. No translation available.
AF163762 mRNA. Translation: AAG35563.1.
CCDSiCCDS12206.1. [Q9H324-1]
CCDS62529.1. [Q9H324-2]
RefSeqiNP_001269281.1. NM_001282352.1. [Q9H324-2]
NP_112219.3. NM_030957.3.
UniGeneiHs.657508.

Genome annotation databases

EnsembliENST00000595838; ENSP00000470501; ENSG00000142303. [Q9H324-2]
GeneIDi81794.
KEGGihsa:81794.
UCSCiuc002mki.3. human. [Q9H324-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC092315 Genomic DNA. No translation available.
AC130469 Genomic DNA. No translation available.
AF163762 mRNA. Translation: AAG35563.1.
CCDSiCCDS12206.1. [Q9H324-1]
CCDS62529.1. [Q9H324-2]
RefSeqiNP_001269281.1. NM_001282352.1. [Q9H324-2]
NP_112219.3. NM_030957.3.
UniGeneiHs.657508.

3D structure databases

ProteinModelPortaliQ9H324.
SMRiQ9H324.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123586. 7 interactors.
IntActiQ9H324. 7 interactors.
MINTiMINT-8247349.
STRINGi9606.ENSP00000270328.

Protein family/group databases

MEROPSiM12.235.

PTM databases

iPTMnetiQ9H324.
PhosphoSitePlusiQ9H324.

Polymorphism and mutation databases

DMDMi148887344.

Proteomic databases

PaxDbiQ9H324.
PeptideAtlasiQ9H324.
PRIDEiQ9H324.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000595838; ENSP00000470501; ENSG00000142303. [Q9H324-2]
GeneIDi81794.
KEGGihsa:81794.
UCSCiuc002mki.3. human. [Q9H324-1]

Organism-specific databases

CTDi81794.
DisGeNETi81794.
GeneCardsiADAMTS10.
GeneReviewsiADAMTS10.
HGNCiHGNC:13201. ADAMTS10.
HPAiHPA040223.
MalaCardsiADAMTS10.
MIMi277600. phenotype.
608990. gene.
neXtProtiNX_Q9H324.
OpenTargetsiENSG00000142303.
Orphaneti3449. Weill-Marchesani syndrome.
PharmGKBiPA24537.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3538. Eukaryota.
ENOG410XPKZ. LUCA.
GeneTreeiENSGT00760000118880.
HOGENOMiHOG000004800.
HOVERGENiHBG004315.
InParanoidiQ9H324.
KOiK08625.
PhylomeDBiQ9H324.
TreeFamiTF313537.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000142303-MONOMER.
ReactomeiR-HSA-5083635. Defective B3GALTL causes Peters-plus syndrome (PpS).
R-HSA-5173214. O-glycosylation of TSR domain-containing proteins.

Miscellaneous databases

GeneWikiiADAMTS10.
GenomeRNAii81794.
PROiQ9H324.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000142303.
CleanExiHS_ADAMTS10.
ExpressionAtlasiQ9H324. baseline and differential.
GenevisibleiQ9H324. HS.

Family and domain databases

Gene3Di3.40.390.10. 1 hit.
InterProiIPR010294. ADAM_spacer1.
IPR024079. MetalloPept_cat_dom.
IPR001590. Peptidase_M12B.
IPR013273. Peptidase_M12B_ADAM-TS.
IPR002870. Peptidase_M12B_N.
IPR010909. PLAC.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF05986. ADAM_spacer1. 1 hit.
PF01562. Pep_M12B_propep. 1 hit.
PF08686. PLAC. 1 hit.
PF01421. Reprolysin. 1 hit.
PF00090. TSP_1. 5 hits.
[Graphical view]
PRINTSiPR01857. ADAMTSFAMILY.
SMARTiSM00209. TSP1. 5 hits.
[Graphical view]
SUPFAMiSSF82895. SSF82895. 5 hits.
PROSITEiPS50215. ADAM_MEPRO. 1 hit.
PS50900. PLAC. 1 hit.
PS50092. TSP1. 5 hits.
PS00142. ZINC_PROTEASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiATS10_HUMAN
AccessioniPrimary (citable) accession number: Q9H324
Secondary accession number(s): M0QZE4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 5, 2001
Last sequence update: June 12, 2007
Last modified: November 2, 2016
This is version 146 of the entry and version 2 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 19
    Human chromosome 19: 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
  5. Peptidase families
    Classification of peptidase families and list of entries
  6. 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.