| Post Translational Modifications | The precursor is processed by a furin endopeptidase which cleaves off the pro-domain. O-glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion. |
| Function | Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation. |
| Protein Name | A Disintegrin And Metalloproteinase With Thrombospondin Motifs 13Adam-Ts 13Adam-Ts13Adamts-13Von Willebrand Factor-Cleaving ProteaseVwf-CpVwf-Cleaving Protease |
| Database Links | Reactome: R-MMU-5173214Reactome: -MMU-75892 |
| Cellular Localisation | SecretedSecretion Enhanced By O-Fucosylation Of Tsp Type-1 Repeats |
| Alternative Protein Names | A Disintegrin And Metalloproteinase With Thrombospondin Motifs 13 proteinAdam-Ts 13 proteinAdam-Ts13 proteinAdamts-13 proteinVon Willebrand Factor-Cleaving Protease proteinVwf-Cp proteinVwf-Cleaving Protease proteinAdamts13 proteinGm710 protein |
Information sourced from Uniprot.org
