Cloned (Comment) | Organism |
---|---|
gene Adamts13, DNA and amino acid sequence determination and analysis, expression of truncated ADAMTS13 in 129/Sv transgenic mice | Mus musculus |
Protein Variants | Comment | Organism |
---|---|---|
additional information | generation of a congenic mouse model expressing the C-terminally truncated form of ADAMTS13 on 129/Sv genetic background, presence of IAP insertion in the Adamts13 gene of the congenic Adamts13S/S mice by PCR, and detection an IAP chimeric transcript by Northern blotting of RNA from liver, overview. The distal C-terminally truncated form of mouseADAMTS13 does not completely lose the activity. In vivo thrombus growth is accelerated in Adamts13S/S mice, overview | Mus musculus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | secreted enzyme | Mus musculus | - |
- |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | Mus musculus | - |
? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
129/Sv mice | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
liver | primary site of ADAMTS13 synthesis, the enzyme is secreted | Mus musculus | - |
plasma | - |
Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
FRET-VWF73 + H2O | 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 | Mus musculus | ? | - |
? | |
GST-VWF73 + H2O | 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 | Mus musculus | ? | - |
? | |
von Willebrand factor + H2O | - |
Mus musculus | ? | - |
? | |
von Willebrand factor + H2O | specific cleavage of ultra-large von Willebrand factor multimers | Mus musculus | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Mus musculus |
General Information | Comment | Organism |
---|---|---|
physiological function | ADAMTS13 is a multidomain protease that limits platelet thrombogenesis through the cleavage of von Willebrand factor and contributes to inhibition of platelet aggregation. Accelerated thrombus growth occurs in truncated mutant Adamts13S/S mice compared with wild-type full-length Adamts13L/L mice, indicating that the distal C-terminally truncated form of mouse ADAMTS13 has significantly reduced activity in vivo | Mus musculus |