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ADAMTS
ADAMTS (short for a disintegrin and metalloproteinase with thrombospondin motifs) is a family of multidomain extracellular protease enzymes. 19 members of this family have been identified in humans, the first of which, ADAMTS1, was described in 1997. Known functions of the ADAMTS proteases include processing of procollagens and von Willebrand factor as well as cleavage of aggrecan, versican, brevican and neurocan, making them key remodeling enzymes of the extracellular matrix. They have been demonstrated to have important roles in connective tissue organization, coagulation, inflammation, arthritis, angiogenesis and cell migration. Homologous subfamily of ADAMTSL (ADAMTS-like) proteins, which lack enzymatic activity, has also been described. Most cases of thrombotic thrombocytopenic purpura arise from autoantibody-mediated inhibition of ADAMTS13. Like A disintegrin and metalloproteinase, ADAMs, the name of the ADAMTS family refers to its disintegrin and metalloproteinase activity, an ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thrombotic Thrombocytopenic Purpura
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in thrombi, blood clots forming in small blood vessels throughout the body. This results in a thrombocytopenia, low platelet count, hemolytic anemia, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction. Symptoms may include ecchymosis, large bruises, fever, weakness, shortness of breath, confusion, and headache. Repeated episodes may occur. In about half of cases a trigger is identified, while in the remainder the cause remains unknown. Known triggers include bacterial infections, certain medications, autoimmune diseases such as Systemic lupus erythematosus, lupus, and pregnancy. The underlying mechanism typically involves antibody, antibodies inhibiting the enzyme ADAMTS13. This results in decreased break down of large multimers of von Willebrand factor (vWF) into smaller units. Less commonly TTP is heredity, inherited, known as Upshaw–Schulman syndrome, such that A ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |