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Literature summary for 3.4.24.87 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
Search for more literature for 3.4.24.87

Cloned(Commentary)

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

Protein Variants

Protein Variants Comment Organism
E663A site-directed mutagenesis Homo sapiens
E664A site-directed mutagenesis Homo sapiens
G662A site-directed mutagenesis Homo sapiens
R659A site-directed mutagenesis Homo sapiens
R660A site-directed mutagenesis Homo sapiens
R660A/Y661A site-directed mutagenesis Homo sapiens
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
R660A/Y665A site-directed mutagenesis Homo sapiens
Y658A site-directed mutagenesis Homo sapiens
Y661A site-directed mutagenesis Homo sapiens
Y661A/Y665A site-directed mutagenesis Homo sapiens
Y665A site-directed mutagenesis Homo sapiens

Metals/Ions

Metals/Ions Comment Organism Structure
Ca2+ required Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
von Willebrand factor + H2O Homo sapiens
-
 ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

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

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
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 ?
-
 ?
von Willebrand factor + H2O
-
 Homo sapiens ?
-
 ?
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

Synonyms Comment Organism
ADAMTS13
-
 Homo sapiens

Temperature Optimum [°C]

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

pH Optimum

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

General Information

General Information Comment Organism
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