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Protein

A disintegrin and metalloproteinase with thrombospondin motifs 19

Gene

ADAMTS19

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript leveli

Functioni

Cofactori

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi294Zinc; in inhibited formBy similarity1
Metal bindingi482Zinc; catalyticBy similarity1
Active sitei483PROSITE-ProRule annotation1
Metal bindingi486Zinc; catalyticBy similarity1
Metal bindingi492Zinc; catalyticBy similarity1

GO - Molecular functioni

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Metal-binding, Zinc

Enzyme and pathway databases

BioCyciZFISH:ENSG00000145808-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

MEROPSiM12.029.

Names & Taxonomyi

Protein namesi
Recommended name:
A disintegrin and metalloproteinase with thrombospondin motifs 19 (EC:3.4.24.-)
Short name:
ADAM-TS 19
Short name:
ADAM-TS19
Short name:
ADAMTS-19
Gene namesi
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:17111. ADAMTS19.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Extracellular matrix, Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi171019.
OpenTargetsiENSG00000145808.
PharmGKBiPA24545.

Polymorphism and mutation databases

BioMutaiADAMTS19.
DMDMi296434402.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 21Sequence analysisAdd BLAST21
PropeptideiPRO_000002920222 – 316By similarityAdd BLAST295
ChainiPRO_0000029203317 – 1207A disintegrin and metalloproteinase with thrombospondin motifs 19Add BLAST891

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi260N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi401 ↔ 466By similarity
Disulfide bondi441 ↔ 448By similarity
Disulfide bondi460 ↔ 540By similarity
Disulfide bondi499 ↔ 524By similarity
Disulfide bondi569 ↔ 593By similarity
Disulfide bondi580 ↔ 601By similarity
Disulfide bondi588 ↔ 620By similarity
Disulfide bondi614 ↔ 625By similarity
Disulfide bondi645 ↔ 680By similarity
Disulfide bondi649 ↔ 685By similarity
Disulfide bondi660 ↔ 670By similarity
Glycosylationi797N-linked (GlcNAc...)Sequence analysis1
Glycosylationi907N-linked (GlcNAc...)Sequence analysis1
Glycosylationi949N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi988 ↔ 1031By similarity
Disulfide bondi992 ↔ 1036By similarity
Disulfide bondi1003 ↔ 1020By similarity
Glycosylationi1009N-linked (GlcNAc...)Sequence analysis1

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-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

EPDiQ8TE59.
PaxDbiQ8TE59.
PeptideAtlasiQ8TE59.
PRIDEiQ8TE59.

PTM databases

iPTMnetiQ8TE59.
PhosphoSitePlusiQ8TE59.

Expressioni

Tissue specificityi

Expressed in fetal lung, but not in any adult tissues examined. Expression was detected in an osteosarcoma cDNA library.

Gene expression databases

BgeeiENSG00000145808.
CleanExiHS_ADAMTS19.
ExpressionAtlasiQ8TE59. baseline and differential.
GenevisibleiQ8TE59. HS.

Organism-specific databases

HPAiHPA056171.
HPA058438.

Structurei

3D structure databases

ProteinModelPortaliQ8TE59.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini325 – 545Peptidase M12BPROSITE-ProRule annotationAdd BLAST221
Domaini546 – 633DisintegrinAdd BLAST88
Domaini634 – 686TSP type-1 1PROSITE-ProRule annotationAdd BLAST53
Domaini915 – 975TSP type-1 2PROSITE-ProRule annotationAdd BLAST61
Domaini976 – 1037TSP type-1 3PROSITE-ProRule annotationAdd BLAST62
Domaini1039 – 1083TSP type-1 4PROSITE-ProRule annotationAdd BLAST45
Domaini1087 – 1144TSP type-1 5PROSITE-ProRule annotationAdd BLAST58
Domaini1160 – 1199PLACPROSITE-ProRule annotationAdd BLAST40

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni791 – 914SpacerAdd BLAST124

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi292 – 299Cysteine switchBy similarity8

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi88 – 216Pro-richAdd BLAST129
Compositional biasi687 – 790Cys-richAdd BLAST104

Domaini

The conserved cysteine present in the cysteine-switch motif binds the catalytic zinc ion, thus inhibiting the enzyme. The dissociation of the cysteine from the zinc ion upon the activation-peptide release activates the enzyme.

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.
HOVERGENiHBG050621.
InParanoidiQ8TE59.
KOiK08633.
OMAiKECKTKL.
OrthoDBiEOG091G14M8.
PhylomeDBiQ8TE59.
TreeFamiTF313537.

Family and domain databases

Gene3Di3.40.390.10. 1 hit.
InterProiIPR006586. ADAM_Cys-rich.
IPR010294. 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.
SMARTiSM00608. ACR. 1 hit.
SM00209. 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]

Sequencei

Sequence statusi: Complete.

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

Q8TE59-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MRLTHICCCC LLYQLGFLSN GIVSELQFAP DREEWEVVFP ALWRREPVDP
60 70 80 90 100
AGGSGGSADP GWVRGVGGGG SARAQAAGSS REVRSVAPVP LEEPVEGRSE
110 120 130 140 150
SRLRPPPPSE GEEDEELESQ ELPRGSSGAA ALSPGAPASW QPPPPPQPPP
160 170 180 190 200
SPPPAQHAEP DGDEVLLRIP AFSRDLYLLL RRDGRFLAPR FAVEQRPNPG
210 220 230 240 250
PGPTGAASAP QPPAPPDAGC FYTGAVLRHP GSLASFSTCG GGLMGFIQLN
260 270 280 290 300
EDFIFIEPLN DTMAITGHPH RVYRQKRSME EKVTEKSALH SHYCGIISDK
310 320 330 340 350
GRPRSRKIAE SGRGKRYSYK LPQEYNIETV VVADPAMVSY HGADAARRFI
360 370 380 390 400
LTILNMVFNL FQHKSLSVQV NLRVIKLILL HETPPELYIG HHGEKMLESF
410 420 430 440 450
CKWQHEEFGK KNDIHLEMST NWGEDMTSVD AAILITRKDF CVHKDEPCDT
460 470 480 490 500
VGIAYLSGMC SEKRKCIIAE DNGLNLAFTI AHEMGHNMGI NHDNDHPSCA
510 520 530 540 550
DGLHIMSGEW IKGQNLGDVS WSRCSKEDLE RFLRSKASNC LLQTNPQSVN
560 570 580 590 600
SVMVPSKLPG MTYTADEQCQ ILFGPLASFC QEMQHVICTG LWCKVEGEKE
610 620 630 640 650
CRTKLDPPMD GTDCDLGKWC KAGECTSRTS APEHLAGEWS LWSPCSRTCS
660 670 680 690 700
AGISSRERKC PGLDSEARDC NGPRKQYRIC ENPPCPAGLP GFRDWQCQAY
710 720 730 740 750
SVRTSSPKHI LQWQAVLDEE KPCALFCSPV GKEQPILLSE KVMDGTSCGY
760 770 780 790 800
QGLDICANGR CQKVGCDGLL GSLAREDHCG VCNGNGKSCK IIKGDFNHTR
810 820 830 840 850
GAGYVEVLVI PAGARRIKVV EEKPAHSYLA LRDAGKQSIN SDWKIEHSGA
860 870 880 890 900
FNLAGTTVHY VRRGLWEKIS AKGPTTAPLH LLVLLFQDQN YGLHYEYTIP
910 920 930 940 950
SDPLPENQSS KAPEPLFMWT HTSWEDCDAT CGGGERKTTV SCTKIMSKNI
960 970 980 990 1000
SIVDNEKCKY LTKPEPQIRK CNEQPCQTRW MMTEWTPCSR TCGKGMQSRQ
1010 1020 1030 1040 1050
VACTQQLSNG TLIRARERDC IGPKPASAQR CEGQDCMTVW EAGVWSECSV
1060 1070 1080 1090 1100
KCGKGIRHRT VRCTNPRKKC VLSTRPREAE DCEDYSKCYV WRMGDWSKCS
1110 1120 1130 1140 1150
ITCGKGMQSR VIQCMHKITG RHGNECFSSE KPAAYRPCHL QPCNEKINVN
1160 1170 1180 1190 1200
TITSPRLAAL TFKCLGDQWP VYCRVIREKN LCQDMRWYQR CCETCRDFYA

QKLQQKS
Length:1,207
Mass (Da):134,048
Last modified:May 18, 2010 - v2
Checksum:i21EFB26660DEB6D9
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti367S → G in CAC84565 (PubMed:11867212).Curated1
Sequence conflicti1048C → F in CAC84565 (PubMed:11867212).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_036154360L → I in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_057087582E → G.Corresponds to variant rs10062501dbSNPEnsembl.1
Natural variantiVAR_0245991089Y → F.Corresponds to variant rs11749126dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ311904 mRNA. Translation: CAC84565.1.
AC008425 Genomic DNA. No translation available.
AC008528 Genomic DNA. No translation available.
AC008591 Genomic DNA. No translation available.
AC106781 Genomic DNA. No translation available.
AC129713 Genomic DNA. No translation available.
RefSeqiNP_598377.4. NM_133638.4.
UniGeneiHs.23751.

Genome annotation databases

EnsembliENST00000274487; ENSP00000274487; ENSG00000145808.
GeneIDi171019.
KEGGihsa:171019.
UCSCiuc003kvb.2. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ311904 mRNA. Translation: CAC84565.1.
AC008425 Genomic DNA. No translation available.
AC008528 Genomic DNA. No translation available.
AC008591 Genomic DNA. No translation available.
AC106781 Genomic DNA. No translation available.
AC129713 Genomic DNA. No translation available.
RefSeqiNP_598377.4. NM_133638.4.
UniGeneiHs.23751.

3D structure databases

ProteinModelPortaliQ8TE59.
ModBaseiSearch...
MobiDBiSearch...

Protein family/group databases

MEROPSiM12.029.

PTM databases

iPTMnetiQ8TE59.
PhosphoSitePlusiQ8TE59.

Polymorphism and mutation databases

BioMutaiADAMTS19.
DMDMi296434402.

Proteomic databases

EPDiQ8TE59.
PaxDbiQ8TE59.
PeptideAtlasiQ8TE59.
PRIDEiQ8TE59.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000274487; ENSP00000274487; ENSG00000145808.
GeneIDi171019.
KEGGihsa:171019.
UCSCiuc003kvb.2. human.

Organism-specific databases

CTDi171019.
DisGeNETi171019.
GeneCardsiADAMTS19.
H-InvDBHIX0024810.
HGNCiHGNC:17111. ADAMTS19.
HPAiHPA056171.
HPA058438.
MIMi607513. gene.
neXtProtiNX_Q8TE59.
OpenTargetsiENSG00000145808.
PharmGKBiPA24545.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3538. Eukaryota.
ENOG410XPKZ. LUCA.
GeneTreeiENSGT00760000118880.
HOGENOMiHOG000004800.
HOVERGENiHBG050621.
InParanoidiQ8TE59.
KOiK08633.
OMAiKECKTKL.
OrthoDBiEOG091G14M8.
PhylomeDBiQ8TE59.
TreeFamiTF313537.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000145808-MONOMER.
ReactomeiR-HSA-5083635. Defective B3GALTL causes Peters-plus syndrome (PpS).
R-HSA-5173214. O-glycosylation of TSR domain-containing proteins.

Miscellaneous databases

ChiTaRSiADAMTS19. human.
GenomeRNAii171019.
PROiQ8TE59.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000145808.
CleanExiHS_ADAMTS19.
ExpressionAtlasiQ8TE59. baseline and differential.
GenevisibleiQ8TE59. HS.

Family and domain databases

Gene3Di3.40.390.10. 1 hit.
InterProiIPR006586. ADAM_Cys-rich.
IPR010294. 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.
SMARTiSM00608. ACR. 1 hit.
SM00209. 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 nameiATS19_HUMAN
AccessioniPrimary (citable) accession number: Q8TE59
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 25, 2003
Last sequence update: May 18, 2010
Last modified: November 2, 2016
This is version 133 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 5
    Human chromosome 5: 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.