Biomaterial Database

Pathogenesis of thrombotic thrombocytopenic purpura. 2003

Thomas J Raife

Department of Pathology, C250 GH, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA. thomas-raife@uiowa.edu

The recent discovery of important molecular and genetic mechanisms of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome provide an opportunity to reconstruct scientific and clinical paradigms. Acquired and congenital defects of the metalloprotease ADAMTS13 are a central feature in the pathogenesis of a major type of thrombotic microangiopathy. This and other pathogenic mechanisms can redefine the terminology of thrombotic microangiopathy. Deficient activity of ADAMTS13 suggests several possible models of microvascular thrombosis. The sporadic relationship between thrombotic microangiopathy and ADAMTS13 deficiency draws attention to other critical pathologic factors that are still poorly understood. Investigation of vascular injury and mechanisms of microvascular thrombosis remain the frontiers of investigation in thrombotic microangiopathy.

UI	MeSH Term	Description	Entries
D008666	Metalloendopeptidases	ENDOPEPTIDASES which use a metal such as ZINC in the catalytic mechanism.	Metallo-Endoproteinases,Metalloendopeptidase
D008833	Microcirculation	The circulation of the BLOOD through the MICROVASCULAR NETWORK.	Microvascular Blood Flow,Microvascular Circulation,Blood Flow, Microvascular,Circulation, Microvascular,Flow, Microvascular Blood,Microvascular Blood Flows,Microvascular Circulations
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D001792	Blood Platelets	Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.	Platelets,Thrombocytes,Blood Platelet,Platelet,Platelet, Blood,Platelets, Blood,Thrombocyte
D006463	Hemolytic-Uremic Syndrome	A syndrome that is associated with microvascular diseases of the KIDNEY, such as RENAL CORTICAL NECROSIS. It is characterized by hemolytic anemia (ANEMIA, HEMOLYTIC); THROMBOCYTOPENIA; and ACUTE RENAL FAILURE.	Gasser's Syndrome,Gasser Syndrome,Gassers Syndrome,Hemolytic Uremic Syndrome,Syndrome, Hemolytic-Uremic
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000071120	ADAMTS13 Protein	An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA.	A Disintegrin and Metalloproteinase with Thrombospondin Motifs 13 Protein,ADAMTS-13 Protein,ADAMTS13 Protease,vWF-Cleaving Protease,von Willebrand Factor-Cleaving Protease,ADAMTS 13 Protein,vWF Cleaving Protease,von Willebrand Factor Cleaving Protease
D014841	von Willebrand Factor	A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in VON WILLEBRAND DISEASES is due to the deficiency of this factor.	Factor VIII-Related Antigen,Factor VIIIR-Ag,Factor VIIIR-RCo,Plasma Factor VIII Complex,Ristocetin Cofactor,Ristocetin-Willebrand Factor,von Willebrand Protein,Factor VIII Related Antigen,Factor VIIIR Ag,Factor VIIIR RCo,Ristocetin Willebrand Factor
D051722	ADAM Proteins	A family of membrane-anchored glycoproteins that contain a disintegrin and metalloprotease domain. They are responsible for the proteolytic cleavage of many transmembrane proteins and the release of their extracellular domain.	A Disintegrin and Metalloprotease Protein,A Disintegrin and Metalloprotease Proteins,ADAM (A Disintegrin and Metalloprotease) Proteins