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Literature summary for 3.4.24.87 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

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

Molecular Weight [Da]

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

Source Tissue

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

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
von Willebrand factor + H2O
-
Homo sapiens ?
-
?
von Willebrand factor + H2O substrate is FRET-VWF73 Homo sapiens ?
-
?

Subunits

Subunits Comment Organism
? x * 150000, SDS-PAGE, x * 190000, non-reducing PAGE Homo sapiens
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

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

General Information

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