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Literature summary extracted from

  • Ling, J.; Ma, Z.; Liu, L.; Yin, J.; Su, J.; Shen, F.; Xie, L.; Hu, S.
    Identification of a crucial tryptophan residue in ADAMTS13 required for its secretion and enzymatic activity (2018), Clin. Exp. Pharmacol. Physiol., 45, 1181-1186 .
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 W390A the mutant has impaired binding affinity to its substrate von Willebrand factor. The mutation retards the enzyme's secretion, leading to its deposition in endoplasmic reticulum. Compared with the wild type enzyme, the mutant also has a decreased cleavage activity for multimeric von Willebrand factor under both static and shear stress conditions 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 Q76LX8
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 HeLa cell
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Subunits

EC Number Subunits Comment Organism
3.4.24.87 ? x * 190000, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 a disintegrin and metalloprotease with thrombospondin motifs 13
-
 Homo sapiens
3.4.24.87 ADAMTS13
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 malfunction functional deficiency of the enzyme causally assists in the pathology of thrombotic thrombocytopenic purpura Homo sapiens