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20-September-2008 09:55:52 - ADAMTS13 ADAM metallopeptidase with thrombospondin type 1 motif, 13 Identifiers Symbols ADAMTS13; TTP; C9orf8; DKFZp434C2322; FLJ42993; MGC118899; MGC118900; VWFCP; vWF-CP External IDs OMIM: 604134 MGI: 2685556 HomoloGene: 16372 Gene ontology Molecular function: metalloendopeptidase activity integrin binding calcium ion binding protein binding peptidase activity zinc ion binding Cellular component: proteinaceous extracellular matrix extracellular space cell surface Biological process: proteolysis cell-matrix adhesion integrin-mediated signaling pathway glycoprotein metabolic process protein processing platelet activation peptide catabolic process RNA expression pattern More reference expression data Orthologs Human Mouse Entrez 11093 279028 Ensembl ENSG00000160323 n/a Uniprot Q76LX8 n/a Refseq NM_139028 mRNA NP_620597 protein XM_914175 mRNA XP_919268 protein Location Chr 9: 135.28 - 135.31 Mb n/a Pubmed search 1 2 ADAMTS13 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13-also known as von Willebrand factor-cleaving protease VWFCP-is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor vWf, a large protein involved in blood clotting. It is secreted in blood and degrades large vWf multimers, decreasing their activity.1 Contents 1 Genetics 2 Discovery and function 3 Proteomics 4 Role in disease 5 See also 6 References 7 Further reading 8 External links Genetics The ADAMTS13 gene maps to the ninth chromosome 9q34.1 Discovery and function Since 1982 it had been known that thrombotic thrombocytopenic purpura TTP, one of the microangiopathic hemolytic anemias see below, was characterised in its familial form by the presence in plasma of unusually large von Willebrand factor multimers ULVWF.1 In 1994, VWF was shown to be cleaved between a tyrosine at position 1605 and a methionine at 1606 by a plasma metalloprotease when it was exposed to high levels of shear stress. In 1996, two research groups independently further characterized the enzyme that cleaved VWF. In the next two years, the same two groups showed that the congenital deficiency of vWf-cleaving protease was associated with formation of platelet microthrombi in the small blood vessels. In addition, they reported that in majority of patients IgG antibodies, directed against this enzyme, caused TTP in non-familial cases.1 Proteomics Genomically, ADAMTS13 shares many properties with the 19 member ADAMTS family, all of which are characterised by a protease domain the part that performs the protein hydrolysis, an adjacent disintegrin domain and one or more thrombospondin domains. ADAMTS13 in fact has eight thrombospondin domains. It has no hydrophobic transmembrane domain, and hence it not anchored in the cell membrane.1 Role in disease Deficiency of ADAMTS13 was originally discovered in Upshaw-Shulman syndrome, the recurring familial form of TTP. By that time it was already suspected that TTP occurred in the autoimmune form as well, owing to its response to plasmapheresis and characterisation of IgG inhibitors. Since the discovery of ADAMTS13, specific epitopes on its surface have been shown to be the target of inhibitory antibodies.123 Especially since the link between aortic valve stenosis and angiodysplasia was proven to be due to high shear stress Heyde's syndrome, it has been accepted that increased exposure of vWf to ADAMTS13 due to various reasons would predispose to bleeding by causing increased degradation of vWf. This phenomenon is characterised by a form of von Willebrand disease type 2a.1 See also ADAMTS5 References ^ a b c d e f g Levy GG, Motto DG, Ginsburg D 2005. ADAMTS13 turns 3. Blood 106 1: 11-7. doi:10.1182/blood-2004-10-4097. PMID 15774620. ^ Tsai HM 2003. Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. J. Am. Soc. Nephrol. 14 4: 1072-81. doi:10.1097/01.ASN.0000060805.04118.4C. PMID 12660343. ^ Furlan M, Lämmle B 2001. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol 14 2: 437-54. doi:10.1053/beha.2001.0142. PMID 11686108. Further reading Furlan M, Lammle B. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol 2001;14:437-54. PMID 11686108. Tsai HM. Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. J Am Soc Nephrol 2003;14:1072-81. PMID 12660343. Tang BL 2001. ADAMTS: a novel family of extracellular matrix proteases.. Int. J. Biochem. Cell Biol. 33 1: 33-44. PMID 11167130. Fujimura Y, Matsumoto M, Yagi H, et al. 2002. Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome.. Int. J. Hematol. 75 1: 25-34. PMID 11843286. Zheng X, Majerus EM, Sadler JE 2003. ADAMTS13 and TTP.. Curr. Opin. Hematol. 9 5: 389-94. PMID 12172456. Tsai HM 2003. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.. J. Mol. Med. 80 10: 639-47. doi:10.1007/s00109-002-0369-8. PMID 12395148. Tsai HM 2003. Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura.. Arterioscler. Thromb. Vasc. Biol. 23 3: 388-96. doi:10.1161/01.ATV.0000058401.34021.D4. PMID 12615692. Tsai HM 2003. Is severe deficiency of ADAMTS-13 specific for thrombotic thrombocytopenic purpura? Yes.. J. Thromb. Haemost. 1 4: 625-31. PMID 12871390. Remuzzi G 2003. Is ADAMTS-13 deficiency specific for thrombotic thrombocytopenic purpura? No.. J. Thromb. Haemost. 1 4: 632-4. PMID 12871391. Moake JL 2004. von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura.. Semin. Hematol. 41 1: 4-14. PMID 14727254. López JA, Dong JF 2004. Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells.. Semin. Hematol. 41 1: 15-23. PMID 14727255. Plaimauer B, Scheiflinger F 2004. Expression and characterization of recombinant human ADAMTS-13.. Semin. Hematol. 41 1: 24-33. PMID 14727256. Kokame K, Miyata T 2004. Genetic defects leading to herary thrombotic thrombocytopenic purpura.. Semin. Hematol. 41 1: 34-40. PMID 14727257. Schneppenheim R, Budde U, Hassenpflug W, Obser T 2004. Severe ADAMTS-13 deficiency in childhood.. Semin. Hematol. 41 1: 83-9. PMID 14727263. Kremer Hovinga JA, Studt JD, Lämmle B 2005. The von Willebrand factor-cleaving protease ADAMTS-13 and the diagnosis of thrombotic thrombocytopenic purpura TTP.. Pathophysiol. Haemost. Thromb. 33 5-6: 417-21. doi:10.1159/000083839. PMID 15692254. Levy GG, Motto DG, Ginsburg D 2005. ADAMTS13 turns 3.. Blood 106 1: 11-7. doi:10.1182/blood-2004-10-4097. PMID 15774620. George JN 2005. ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome.. Curr. Hematol. Rep. 4 3: 167-9. PMID 15865866. Dong JF 2005. Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions.. J. Thromb. Haemost. 3 8: 1710-6. doi:10.1111/j.1538-7836.2005.01360.x. PMID 16102037. External links Online 'Mendelian Inheritance in Man' OMIM 274150 Secreted protein database entry v d e Proteases: metalloendopeptidases EC 3.4.24 ADAM proteins Alpha secretases ADAM9 · ADAM10 · ADAM17 · ADAM19 · ADAM2 · ADAM7 · ADAM8 · ADAM11 · ADAM12 · ADAM15 · ADAM18 · ADAM22 · ADAM23 · ADAM28 · ADAM33 · ADAMTS1 · ADAMTS2 · ADAMTS3 · ADAMTS4 · ADAMTS5 · ADAMTS8 · ADAMTS9 · ADAMTS10 · ADAMTS12 · ADAMTS13 Matrix metalloproteinase Collagenase · Gelatinase Other Neprilysin · Procollagen peptidase · Thermolysin · Pregnancy-associated plasma protein A · Bone morphogenetic protein 1 · Insulysin · Lysostaphin · Insulin degrading enzyme Retrieved from http://en..org/wiki/ADAMTS13 Categories: Genes on chromosome 9 | Human proteins | EC 3.4.24Hidden category: Protein pages needing a picture Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page This page was last modified on 8 July 2008, at 01:26
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