Literature summary for 3.4.24.87 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 .

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Protein Variants

Protein Variants	Comment	Organism
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)

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	Q76LX8	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
HeLa cell	-	Homo sapiens	-

Substrates and Products (Substrate)

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

Subunits

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

Synonyms

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

General Information

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

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Release 2023.1 (January 2023)