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

BRENDA support

Literature summary extracted from

  • Pos, W.; Crawley, J.T.; Fijnheer, R.; Voorberg, J.; Lane, D.A.; Luken, B.M.
    An autoantibody epitope comprising residues R660, Y661, and Y665 in the ADAMTS13 spacer domain identifies a binding site for the A2 domain of VWF (2010), Blood, 115, 1640-1649.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 expression of wild-type and mutant ADAMTS13 in HEK-293 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 E663A site-directed mutagenesis Homo sapiens
3.4.24.87 E664A site-directed mutagenesis Homo sapiens
3.4.24.87 G662A site-directed mutagenesis Homo sapiens
3.4.24.87 R659A site-directed mutagenesis Homo sapiens
3.4.24.87 R660A site-directed mutagenesis Homo sapiens
3.4.24.87 R660A/Y661A site-directed mutagenesis Homo sapiens
3.4.24.87 R660A/Y661A/Y665A site-directed mutagenesis, the ADAMTS13 variant, i.e. ADAMTS13-RYY, shows a 12fold reduced catalytic efficiency arising from over 25fold reduced substrate binding Homo sapiens
3.4.24.87 R660A/Y665A site-directed mutagenesis Homo sapiens
3.4.24.87 Y658A site-directed mutagenesis Homo sapiens
3.4.24.87 Y661A site-directed mutagenesis Homo sapiens
3.4.24.87 Y661A/Y665A site-directed mutagenesis Homo sapiens
3.4.24.87 Y665A site-directed mutagenesis Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Ca2+ required 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
-
?
-
?

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
3.4.24.87 recombinant wild-type and mutant ADAMTS13 from HEK-293 cells by nickel affinity chromatography Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 additional information binding specificities of wild-type ADAMTS13 and ADAMTS13-RYY to von Willebrand factor115, von Willebrand factor106, and full-length von Willebrand factor, overview Homo sapiens ?
-
?
3.4.24.87 von Willebrand factor + H2O
-
Homo sapiens ?
-
?
3.4.24.87 von Willebrand factor + H2O recombinant substrate stably expressed in HEK293 cells, the ADAMTS13 metalloprotease domain cleaves the von Willebrand factor A2 domain at the Y1605-M1606 scissile bond Homo sapiens ?
-
?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13
-
Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.4.24.87 37
-
assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.4.24.87 7.8
-
assay at Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 additional information auto-antibodies, directed toward the spacer domain of ADAMTS13 involving region Y658-Y665 , cause ADAMTS13 deficiency and, as a result, acquired thrombotic thrombocytopenic purpura Homo sapiens