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A disintegrin & metalloproteinase with thrombospondin type 1 motif 10; ADAMTS-10; ADAM-TS 10; ADAM-TS10; (ADAMTS10)

Function: - interaction with the extracellular matrix Cofactor: binds 1 Zn+2 per subunit (putative) Structure: - spacer domain & the TSP type-1 domains are important for a tight interaction with the extracellular matrix - contains 1 disintegrin domain - contains 1 peptidase M12B domain - contains 1 PLAC domain - contains 5 TSP type-1 domains Compartment: - secreted, extracellular space, extracellular matrix (putative) Expression: widely expressed in adult tissues Pathology: - defects in ADAMTS10 are a cause of the autosomal recessive form of Weill-Marchesani syndrome

General

A disintegrin & metalloproteinase with thrombospondin type 1 motif (ADAMTS) glycoprotein

Properties

SIZE: entity length = 1103 aa MW = 121 kD COMPARTMENT: extracellular compartment MOTIF: signal sequence {1-25} proline-rich region SITE: 58-61 MOTIF: proline residue (SEVERAL) N-glycosylation site {N90} N-glycosylation site {N222} Peptidase M12B {239-457} MOTIF: N-glycosylation site {N323} cysteine residue {C370} MODIFICATION: cysteine residue {C452} Zn+2-binding site SITE: 392-392 glutamate residue {E393} Zn+2-binding site SITE: 396-396 Zn+2-binding site SITE: 402-402 cysteine residue {C409} MODIFICATION: cysteine residue {C436} cysteine residue {C436} MODIFICATION: cysteine residue {C409} cysteine residue {C452} MODIFICATION: cysteine residue {C370} disintegrin domain {460-546} TSP1 module {547-602} MOTIF: cysteine residue {C559} MODIFICATION: cysteine residue {C596} cysteine residue {C563} MODIFICATION: cysteine residue {C601} serine-rich region {568-571} MOTIF: serine residue (SEVERAL) cysteine residue {C574} MODIFICATION: cysteine residue {C586} cysteine residue {C586} MODIFICATION: cysteine residue {C574} cysteine residue {C596} MODIFICATION: cysteine residue {C559} cysteine residue {C601} MODIFICATION: cysteine residue {C563} cysteine-rich region {604-705} Spacer {706-828} MOTIF: N-glycosylation site {N740} N-glycosylation site {N795} TSP1 module {825-883} TSP1 module {884-945} MOTIF: N-glycosylation site {N892} TSP1 module {947-1001} TSP1 module {1003-1058} PLAC {1065-1103}

Database Correlations

OMIM correlations MORBIDMAP 608990 UniProt Q9H324 PFAM correlations Entrez Gene 81794 Kegg hsa:81794

References

  1. UniProt :accession Q9H324
  2. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=ADAMTS10