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

BRENDA support

Literature summary extracted from

  • Kokame, K.; Matsumoto, M.; Soejima, K.; Yagi, H.; Ishizashi, H.; Funato, M.; Tamai, H.; Konno, M.; Kamide, K.; Kawano, Y.; Miyata, T.; Fujimura, Y.
    Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity (2002), Proc. Natl. Acad. Sci. USA, 99, 11902-11907.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 DNA sequence determination and analysis, transient expression of wild-type and mutants as FLAG-tagged enzymes in HeLa cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 C508Y naturally occuring mutant, no secretion of the enzyme to the plasma, possible defects in secretion pathway, or protein folding and stability Homo sapiens
3.4.24.87 additional information Q449stop mutant is efficiently secreted, has a MW of 54 kDa, and shows no activity, detection of naturally occuring mutations in a Japanese family with thrombotic thrombocytopenic pupura, overview Homo sapiens
3.4.24.87 P475S naturally occuring mutant, efficient secretion, reduced activity Homo sapiens
3.4.24.87 Q448E naturally occuring mutant, efficient secretion, fully active Homo sapiens
3.4.24.87 R268P naturally occuring mutant, no secretion of the enzyme to the plasma, possible defects in secretion pathway, or protein folding and stability Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular plasma 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 proteins + H2O Homo sapiens
-
 peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O Homo sapiens rapid physiological process that occurs on endothelial surfaces, reduced activity results in thrombotic thrombocytopenic pupura 2 peptides
-
 ?

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 N-glycosylation Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 liver expression at Homo sapiens
-
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 proteins + H2O
-
 Homo sapiens peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O large multimeric complexes are reduced to smaller ones, cleavage of peptide bond Tyr842-Met843 Homo sapiens 2 peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O rapid physiological process that occurs on endothelial surfaces, reduced activity results in thrombotic thrombocytopenic pupura Homo sapiens 2 peptides
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 ? x * 200000-230000, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13
-
 Homo sapiens
3.4.24.87 M12.241 Merops-ID Homo sapiens
3.4.24.87 von Willebrand factor-cleaving protease
-
 Homo sapiens
3.4.24.87 vWF-cleaving protease
-
 Homo sapiens
3.4.24.87 VWF-CP
-
 Homo sapiens