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Literature summary for 3.4.24.87 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)

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

Protein Variants

Protein Variants Comment Organism
C508Y naturally occuring mutant, no secretion of the enzyme to the plasma, possible defects in secretion pathway, or protein folding and stability Homo sapiens
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
P475S naturally occuring mutant, efficient secretion, reduced activity Homo sapiens
Q448E naturally occuring mutant, efficient secretion, fully active Homo sapiens
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

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 rapid physiological process that occurs on endothelial surfaces, reduced activity results in thrombotic thrombocytopenic pupura 2 peptides
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
glycoprotein N-glycosylation Homo sapiens

Source Tissue

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

Subunits

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

Synonyms

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