Information on EC 3.4.24.87 - ADAMTS13 endopeptidase and Organism(s) Mus musculus and UniProt Accession Q769J6

for references in articles please use BRENDA:EC3.4.24.87
Word Map on EC 3.4.24.87
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
Specify your search results
Select one or more organisms in this record:
This record set is specific for:
Mus musculus
UNIPROT: Q769J6
Show additional data
Do not include text mining results
Include (text mining) results
Include results (AMENDA + additional results, but less precise)


The taxonomic range for the selected organisms is: Mus musculus

The enzyme appears in selected viruses and cellular organisms

EC NUMBER
COMMENTARY hide
3.4.24.87
-
RECOMMENDED NAME
GeneOntology No.
ADAMTS13 endopeptidase
-
CAS REGISTRY NUMBER
COMMENTARY hide
334869-10-2
-
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
UniProt
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
metabolism
-
cyclophilin B knock-out mice have reduced ADAMTS13 levels
physiological function
additional information
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
FRET-VWF73 + H2O
?
show the reaction diagram
-
fluorogenic von Willebrand factor-derived peptide substrate. The distal C-terminal domains of ADAMTS13 are not necessary for the cleavage of the VWF73-based peptide substrate
-
-
?
GST-VWF73 + H2O
?
show the reaction diagram
-
labeled von Willebrand factor-derived peptide substrate. The distal C-terminal domains of ADAMTS13 are not necessary for the cleavage of the VWF73-based peptide substrate
-
-
?
von Willebrand factor + H2O
?
show the reaction diagram
von Willebrand factor + H2O
von Willebrand factor 140-kD fragment + von Willebrand factor 176-kD fragment
show the reaction diagram
von Willebrand factor + H2O
von Willebrand factor fragments
show the reaction diagram
-
ADAMTS13 cleaves von Willebrand factor to smaller less-active forms
-
-
?
additional information
?
-
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
von Willebrand factor + H2O
?
show the reaction diagram
von Willebrand factor + H2O
von Willebrand factor 140-kD fragment + von Willebrand factor 176-kD fragment
show the reaction diagram
-
ADAMTS13 cleaves von Willebrand factor at the Tyr1605-Met1606 bond within the central A2 domain
-
-
?
von Willebrand factor + H2O
von Willebrand factor fragments
show the reaction diagram
-
ADAMTS13 cleaves von Willebrand factor to smaller less-active forms
-
-
?
additional information
?
-
Q769J6
important role for ADAMTS13 in preventing excessive spontaneous Weibel-Palade body secretion, and in the regulation of leukocyte adhesion and extravasation during inflammation
-
-
-
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Ba2+
-
activates
Ca2+
-
activates
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
EDTA
-
-
plasma from patients with thrombotic thrombocytopenic purpurea
-
-
-
additional information
-
ADAMTS-13 activity is evaluated in a model of sepsis induced by cecum ligature and puncture in wild-type and Vwf-/- mice. In wild-type mice, cecum ligature and puncture-induced sepsis elicits a significant ADAMTS-13 decrease, and a strong negative correlation exists between von Willebrand factor, VWF, and ADAMTS-13. In Vwf-/- mice, cecum ligature and puncture also induces severe sepsis, but ADAMTS-13 is not significantly diminished
-
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
-
determination of ADAMTS13 antigen and proteolytic activity in murine plasma, overview
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.5 - 8
-
assay at
8
-
assay at
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
37
-
assay at
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
medium expression of ADAMTS-13
Manually annotated by BRENDA team
-
low expression of ADAMTS-13
Manually annotated by BRENDA team
-
medium expression of ADAMTS-13
Manually annotated by BRENDA team
additional information
-
activity is undetectable in heart, brain, kidney and testis
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expression in HEK-293 cell
-
expression of the murine Adamts-13 cDNA in HEK 293 cells
-
expression of wild-type and mutant enzyme forms in HEK-293 cells and CHO cells
-
gene Adamts13, DNA and amino acid sequence determination and analysis, expression of truncated ADAMTS13 in 129/Sv transgenic mice
-
gene Adamts13, DNA and amino acid sequence determination, construction of self-inactivated lentiviral vector, and expression of functional His-tagged ADAMTS13 cDNA in COS-7 cells
-
ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
-
ADAMTS13 deficiency leads to the fatal disorder thrombotic thrombocytopenic purpura. Cyclosporin A, an inhibitor of cyclophilin B, reduces the secretion of ADAMTS13 and leads to conformational changes in the protein resulting in diminished ADAMTS13 proteolytic activity. Cyclophilin B and ADAMTS13 show a direct, functional interaction
medicine
-
ADAMTS13 may be a useful therapeutic agent for stroke in humans