Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Zheng, X.
    A team player: The disintegrin domain of ADAMTS13 (2009), Blood, 113, 5373-5374.
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 additional information construction of disintegrin domain mutants by site-directed mutagenesis, that exhibit dramatically reduced activity toward the von Willebrand factor fragment VWF115, comprising amino acid residues 1554-1668 of von Willebrand factor. The isolated metalloprotease domain of ADAMTS13 alone is ineffective in cleaving, but if the various noncatalytic domains are incrementally added back, proteolytic activity is gradually restored Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.4.24.87 additional information
-
additional information kinetics of ADAMTS13 disintegrin domain mutants with substrate VWF115, overview Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular
-
Homo sapiens
-
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Zn2+ required, three active sites His and catalytic residues Glu coordinate a catalytic Zn2+ ion Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.4.24.87 von Willebrand factor + H2O Homo sapiens
-
von Willebrand factor 140-kD fragment + von Willebrand factor 176-kD fragment
-
?

Organism

EC Number Organism UniProt Comment Textmining
3.4.24.87 Homo sapiens
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.4.24.87 glycoprotein ADAMTS13 is a multidomain glycoprotein Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 plasma
-
Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 additional information the positively charged Arg349 on ADAMTS13 appears to directly interact with the negatively charged Asp1614 on the von Willebrand factor-A2 domain. This seemingly weak interaction between the disintegrin and VWF-A2 appears to be essential for efficient catalysis of von Willebrand factor under static/denaturing conditions. Molecular modeling of the involvement of the disintegrin domain of ADAMTS13 in von Willebrand factor processing, overview Homo sapiens ?
-
?
3.4.24.87 von Willebrand factor + H2O
-
Homo sapiens von Willebrand factor 140-kD fragment + von Willebrand factor 176-kD fragment
-
?
3.4.24.87 von Willebrand factor + H2O binding of all the proximal noncatalytic domains of ADAMTS13 to von Willebrand factor is necessary to position the active site of ADAMTS13 to the scissile bond, Tyr1605-Met1606, on von Willebrand factor, resulting in productive cleavage. The metalloprotease domain of ADAMTS13 alone is ineffective in cleaving von Willebrand factor, linear relationship between the domains of ADAMTS13 and von Willebrand factor proteolysis. All the proximal noncatalytic domains of ADAMTS13 are required for productive engagement with von Willebrand factor-A2 domain at least under static/denaturing conditions Homo sapiens von Willebrand factor 140-kD fragment + von Willebrand factor 176-kD fragment
-
?
3.4.24.87 VWF115 + H2O a von Willebrand factor-derived peptide substrate, comprising amino acid residues 1554-1668 of von Willebrand factor Homo sapiens ?
-
?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 monomer ADAMTS13 is a multidomain glycoprotein. It consists of numerous domains including a metalloprotease domain, a disintegrin domain, first thrombospondin type 1 repeat, i.e. TSP1, a cysteine-rich domain, and a spacer domain, schematic domain structure model of ADAMTS13, overview Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13 ADAMTS13 is a member of the a disintegrin and metalloprotease with thrombospondin type 1 repeats, i.e. ADAMTS, family Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 additional information thrombotic thrombocytopenic purpura is a potentially fatal illness caused primarily by the absence of plasma ADAMTS13 proteolytic activity, as a result of ADAMTS13 mutations or acquired autoantibodies against ADAMTS13 enzyme Homo sapiens