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Literature summary extracted from

  • Manea, M.; Tati, R.; Karlsson, J.; Békássy, Z.; Karpman, D.
    Biologically active ADAMTS13 is expressed in renal tubular epithelial cells (2010), Pediatr. Nephrol., 25, 87-96.
    View publication on PubMed

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular the enzyme is secreted Homo sapiens
-
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.4.24.87 150000
-
 x * 150000, SDS-PAGE, x * 190000, non-reducing PAGE Homo sapiens
3.4.24.87 190000
-
 x * 150000, SDS-PAGE, x * 190000, non-reducing PAGE 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
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 A-498 cell a renal carcinoma cell line Homo sapiens
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3.4.24.87 epithelial cell primary renal cortical cell cultures, real-time PCR and immunohistochemic analysis, overview Homo sapiens
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3.4.24.87 kidney in the renal cortex Homo sapiens
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3.4.24.87 additional information wide tissue distribution of ADAMTS13 expression Homo sapiens
-
3.4.24.87 plasma ADAMTS13 circulates in plasma Homo sapiens
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3.4.24.87 renal tubule in both proximal and distal in healthy persons and in patients with renal disorders, e.g. tubulopathy Homo sapiens
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3.4.24.87 urine only in urine from patients with renal disorder tubulopathy 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 ?
-
 ?
3.4.24.87 von Willebrand factor + H2O substrate is FRET-VWF73 Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 ? x * 150000, SDS-PAGE, x * 190000, non-reducing PAGE Homo sapiens
3.4.24.87 More ADAMTS13 protease consists of a signal peptide, a short propeptide, the metalloprotease domain, a disintegrin-like domain, a thrombospondin-1 repeat, a Cys-rich domain, a spacer domain, seven additional thrombospondin-1 repeats, and two CUB domains Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 a disintegrin-like and metalloprotease with thrombospondin type-1 motifs 13
-
 Homo sapiens
3.4.24.87 ADAMTS13
-
 Homo sapiens
3.4.24.87 von Willebrand factor cleaving protease
-
 Homo sapiens
3.4.24.87 von Willebrand factor-cleaving protease
-
 Homo sapiens
3.4.24.87 vWF-cleaving protease
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 physiological function ADAMTS13 might regulate, after release by tubuli, the homeostasis in the local microenvironment in renal tubular epithelial cells Homo sapiens