ADAMTS13: Molecular biology and clinical implications

摘要

Objectives: Aim of this work to illustrate the molecular biology and pathobiology of ADAMTS13 and its therapeutic implications. Data Sources: Medline databases (PubMed, Med scape, Science Direct. ) Article Selection: The article studied the molecular biology of ADAMTS13 and its role in diseases and therapy. if the articles did not fulfill the inclusion criteria they were excluded. Data Synthesis: Short reviews were made on ADAMTS13 structure and its role in different diseases and therapy. Conclusion: The knowledge of some mechanistic aspects of ADAMTS13 catalysis and its regulation, the development of sensitive and reliable assays in the clinical diagnostics of TMAs and the nature of modifiers of ADAMTS13 activity on VWF multimers in patients affected by TMAs.