Thrombotic thrombocytopenic purpura (TTP) is a rare blood clotting disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and various degrees of organ damage.A262076 It may be inherited (congenital TTP; cTTP) or acquired due to autoantibodies (immune-mediated TTP; iTTP), although cTTP accounts for <5% of all cases of TTP.A262076 Patients with cTTP have a severe deficiency of a plasma metalloproteinase called ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), which is responsible for cleaving large von Willebrand factor multimers and preventing the formation of microthrombi - this deficiency in ADAMTS13 is responsible for the microangiopathic complications associated with cTTP.A262076 Standard therapy for cTTP involves prophylactic plasma infusions to supplement insufficient ADAMTS13 plasma levels.A262071
Apadamtase alfa, sold under the brand name Adzynma (ADAMTS13, recombinant-krhn), was approved by the FDA in November 2023 for use as an enzyme replacement therapy in patients with cTTP.L48756
Fitur visualisasi ini dikembangkan menggunakan pendekatan Graph Theory untuk memetakan hubungan polifarmasi dan molekuler. Entitas (Obat, Target, Gen) direpresentasikan sebagai Simpul (Nodes), sedangkan hubungan biologisnya sebagai Sisi (Edges).
drugbank-id dan name pada skema XML DrugBank.targets/target yang memuat polipeptida sasaran.gene-name dan varian snp-effects.Tata letak grafik menggunakan algoritma Force-Directed Graph (Barnes-Hut). Model fisika ini menerapkan gaya tolak-menolak antar simpul (Gravitasi: -3000) agar tidak tumpang tindih, serta gaya pegas (Spring: 0.04) pada garis penghubung untuk fleksibilitas interaksi.