Literature summary for 3.4.24.87 extracted from

Soejima, K.; Matsumoto, M.; Kokame, K.; Yagi, H.; Ishizashi, H.; Maeda, H.; Nozaki, C.; Miyata, T.; Fujimura, Y.; Nakagaki, T.
ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage (2003), Blood, 102, 3232-3237.

Search for more literature for 3.4.24.87

Cloned(Commentary)

Cloned (Comment)	Organism
transient expression of wild-type and mutants in HeLa cells as FLAG-tagged proteins	Homo sapiens

Protein Variants

Protein Variants	Comment	Organism
D500E	point mutation in the RGD cysteine-rich domain, unaltered activity compared to the wild-type enzyme	Homo sapiens
additional information	construction of 13 sequential C-terminal truncated mutants, mutants lacking the the cysteine-rich and spacer domain show dramatically reduced or no activity, the other mutants retain their activity, overview	Homo sapiens

Inhibitors

Inhibitors	Comment	Organism	Structure
additional information	inhibitory autoantibodies from plasma of 3 patients with acquired thrombotic thrombocytopenic purpura, epitope mapping	Homo sapiens

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
extracellular	plasma	Homo sapiens	-	-

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
proteins + H2O	Homo sapiens	-	peptides	-	?
von Willebrand factor + H2O	Homo sapiens	cleavage of the peptide bond Tyr842-Met843 within the A2 domain, enzyme deficiency causes lethal thrombotic thrombocytopenic purpura	2 peptides of 140 kDa and 176 kDa	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Reaction

Reaction	Comment	Organism	Reaction ID
The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain	the cysteine-rich/spacer domains are essential for the proteolytic activity	Homo sapiens	a

Source Tissue

Source Tissue	Comment	Organism	Textmining
plasma	-	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
proteins + H2O	-	Homo sapiens	peptides	-	?
von Willebrand factor + H2O	cleavage of the peptide bond Tyr842-Met843 within the A2 domain	Homo sapiens	2 peptides of 140 kDa and 176 kDa	-	?
von Willebrand factor + H2O	cleavage of the peptide bond Tyr842-Met843 within the A2 domain, enzyme deficiency causes lethal thrombotic thrombocytopenic purpura	Homo sapiens	2 peptides of 140 kDa and 176 kDa	-	?

Synonyms

Synonyms	Comment	Organism
ADAMTS-13	-	Homo sapiens
M12.241	Merops-ID	Homo sapiens
von Willebrand factor-cleaving protease	-	Homo sapiens
VWF-CP	-	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
8	-	assay at	Homo sapiens

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Release 2023.1 (January 2023)