1iqd: Difference between revisions

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-[[Image:1iqd.png|left|200px]]
 {{STRUCTURE_1iqd|  PDB=1iqd  |  SCENE=  }}
+===Human Factor VIII C2 Domain complexed to human monoclonal BO2C11 Fab.===
+{{ABSTRACT_PUBMED_11418455}}
-===Human Factor VIII C2 Domain complexed to human monoclonal BO2C11 Fab.===
+==Disease==
+[[http://www.uniprot.org/uniprot/FA8_HUMAN FA8_HUMAN]] Defects in F8 are the cause of hemophilia A (HEMA) [MIM:[http://omim.org/entry/306700 306700]]. A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Note=Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non-functional; i.e. the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.<ref>PMID:3012775</ref><ref>PMID:3122181</ref><ref>PMID:2833855</ref><ref>PMID:2835904</ref><ref>PMID:2499363</ref><ref>PMID:2506948</ref><ref>PMID:2510835</ref><ref>PMID:2495245</ref><ref>PMID:2498882</ref><ref>PMID:2104766</ref><ref>PMID:2105106</ref><ref>PMID:1973901</ref><ref>PMID:2105906</ref><ref>PMID:2106480</ref><ref>PMID:2107542</ref><ref>PMID:1908817</ref><ref>PMID:1908096</ref><ref>PMID:1851341</ref><ref>PMID:1356412</ref><ref>PMID:1639429</ref><ref>PMID:1349567</ref><ref>PMID:1301194</ref><ref>PMID:1301932</ref><ref>PMID:1301960</ref><ref>PMID:8449505</ref><ref>PMID:8322269</ref><ref>PMID:7579394</ref><ref>PMID:7794769</ref><ref>PMID:7759074</ref><ref>PMID:8644728</ref><ref>PMID:8639447</ref><ref>PMID:8759905</ref><ref>PMID:9029040</ref><ref>PMID:9326186</ref><ref>PMID:9341862</ref><ref>PMID:9886318</ref><ref>PMID:9450898</ref><ref>PMID:10215414</ref><ref>PMID:9603440</ref><ref>PMID:9452104</ref><ref>PMID:9792405</ref><ref>PMID:9829908</ref><ref>PMID:9569180</ref><ref>PMID:9569189</ref><ref>PMID:10554831</ref><ref>PMID:10338101</ref><ref>PMID:10408784</ref><ref>PMID:10404764</ref><ref>PMID:10910910</ref><ref>PMID:10910913</ref><ref>PMID:10691849</ref><ref>PMID:10886198</ref><ref>PMID:10800171</ref><ref>PMID:10896236</ref><ref>PMID:10612839</ref><ref>PMID:11410838</ref><ref>PMID:11298607</ref><ref>PMID:11442643</ref><ref>PMID:11442647</ref><ref>PMID:11554935</ref><ref>PMID:11748850</ref><ref>PMID:11341489</ref><ref>PMID:12351418</ref><ref>PMID:12406074</ref><ref>PMID:12199686</ref><ref>PMID:11857744</ref><ref>PMID:12203998</ref><ref>PMID:12325022</ref><ref>PMID:11858487</ref><ref>PMID:12195713</ref><ref>PMID:12930394</ref><ref>PMID:12871415</ref><ref>PMID:12614369</ref><ref>PMID:15682412</ref><ref>PMID:15810915</ref><ref>PMID:16805874</ref><ref>PMID:18184865</ref><ref>PMID:21371196</ref>
-{{ABSTRACT_PUBMED_11418455}}
+==Function==
+[[http://www.uniprot.org/uniprot/FA8_HUMAN FA8_HUMAN]] Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa.
 ==About this Structure==
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 ==Reference==
-<ref group="xtra">PMID:011418455</ref><references group="xtra"/>
+<ref group="xtra">PMID:011418455</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Jacquemin, M.]]