Thrombotic Thrombocytopenia Purpura

The ACTIFLUOR™ ADAMTS13 Activity assay is a fluorescence resonance energy transfer (FRET) assay for the measurement of ADAMTS13 in human plasma. ADAMTS13, also known as von Willebrand Factor (vWF) cleaving protease, is a zinc metalloproteinase that cleaves ultra large vWF multimers (UL-vWF) at the Tyr(1605)-Met(1606) bond located in the A2 region of vWF. Studies have shown that low levels of ADAMTS13 activity are associated with Thrombotic Thrombocytopenia Purpura (TTP), a life-threatening hematological condition characterized by low platelet count, microvascular thrombi, red cell fragmentation, CNS and renal complications. An ADAMTS13 activity level below 5% of normal leads to an accumulation of UL-vWF multimers in plasma which bind to receptors on platelets inducing platelet aggregation and formation of intravascular microthrombi. Congenital TTP is a rare heritable disorder caused by mutations within the ADAMTS13 gene which result in the production of non-functional ADAMTS13 protein. The acquired form of TTP is an autoimmune-like disorder caused by the development of autoantibodies to ADAMTS13 that inhibit enzyme activity. Measurement of the ADAMTS13 activity level is useful in the differential diagnosis of patients with TTP from other thrombocytopenic conditions such as hemolytic uremic syndrome (HUS) and idiopathic thrombocytopenic purpura (ITP).

*Not for sale in the USA

The IMUBIND® ADAMTS13 ELISA is intended for the measurement of ADAMTS13 protein in human plasma. ADAMTS13, also known
as von Willebrand Factor (vWF) cleaving protease, is a zinc metalloproteinase that cleaves ultra large vWF multimers (UL-vWF) at the Tyr(1605) Met(1606) bond located in the A2 region of vWF. Studies have shown that low levels of ADAMTS13 protein activity are associated with Thrombotic Thrombocytopenia Purpura (TTP), a life-threatening hematological condition characterized by low platelet count, microvascular thrombi, red cell fragmentation, CNS and renal complications. An ADAMTS13 activity level below 5% of normal leads to an accumulation of UL-vWF multimers in plasma which bind to receptors on platelets inducing platelet aggregation and formation of intravascular microthrombi. Congenital TTP is a rare heritable disorder caused by mutations within the ADAMTS13 gene which result in the production of non-functional ADAMTS13 protein. The acquired form of TTP is an autoimmune-like disorder caused by the development of autoantibodies to ADAMTS13 that inhibit enzyme activity. Measurement of the ADAMTS13 protein level is useful in the differential diagnosis of patients with TTP from other thrombocytopenic conditions such as hemolytic uremic syndrome (HUS) and idiopathic thrombocytopenic purpura (ITP).

*Not for sale in the USA

The IMUBIND® ADAMTS13 Autoantibody ELISA is intended for the measurement of ADAMTS13 IgG autoantibodies in human plasma. ADAMTS13, also known as von Willebrand Factor (vWF) cleaving protease, is a zinc metalloproteinase that cleaves ultra large vWF multimers (UL-vWF) at the Tyr(1605) Met(1606) bond located in the A2 region of vWF. Studies have shown that low levels of ADAMTS13 activity are associated with Thrombotic Thrombocytopenia Purpura (TTP), a life-threatening hematological condition characterized by low platelet count, microvascular thrombi, red cell fragmentation, CNS and renal complications. An ADAMTS13 activity level below 5% of normal leads to an accumulation of ULvWF multimers in plasma which bind to receptors on platelets inducing platelet aggregation and formation of intravascular microthrombi. Congenital TTP is a rare heritable disorder caused by mutations within the ADAMTS13 gene which result in the production of non-functional ADAMTS13 protein. The acquired form of TTP is an autoimmune-like disorder caused by the development of autoantibodies to ADAMTS13 that inhibit enzyme activity. Measurement of the ADAMTS13 autoantibody level is useful in the differential diagnosis of patients with congenital TTP and acquired TTP, from other thrombocytopenic conditions such as hemolytic uremic syndrome (HUS) and idiopathic thrombocytopenic purpura (ITP), which dictates the course of therapy.

*Not for sale in the USA