R. De Cristofaro

PO129 | CLINICAL BENEFIT OF RECOMBINANT ADAMTS13 IN CTTP ASSOCIATED WITH HOMOZYGOUS PD173G MUTATION AND UNDETECTABLE ACTIVITY

Background. Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare hereditary disorder caused by severe deficiency of the ADAMTS13 enzyme, leading to recurrent thrombotic microangiopathy. We report the case of a 34-year-old male with homozygous missense mutation p.D173G, associated with 0% ADAMTS13 activity, d...

cen-The expanded universe of thrombin: a comprehensive review of its hemostasis-independent roles and structure-function relationships

The expanded universe of thrombin: a comprehensive review of its hemostasis-independent roles and structure-function relationships

The molecular basis of thrombin’s versatility is explored, focusing on its allosteric regulation by sodium ions, its two anion-binding exosites, and its primary signaling mechanism via protease-activated receptors.