Sandbox GGC14: Difference between revisions
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== Function == | == Function == | ||
Fibrinogen is an essential protein in | Fibrinogen is an essential protein in coagulation, which is initiated through either an intrinsic or extrinsic pathway. Both pathways trigger a cascade of reactions that lead to the formation of a blood clot. At some point the protease thrombin is activated, thrombin then converts fibrinogen to fibrin. It does this by cleaving both the <scene name='78/781216/Fribrinopeptide_a_and_b/3'>fibrinopeptide A and B</scene> off of the amino terminus of the alpha and beta chains. The alpha and beta knobs will bind to <scene name='78/781216/Aandb_modules/1'>a and b holes</scene> of other fibrin molecules making fibrin mesh strong enough to hold the platelet plug. | ||
== Disease == | == Disease == | ||
'''Congenital Afibrinogenemia''' – a genetic disorder that results in the lack of fibrinogen which causes abnormal bleeding including gastrointestinal hemorrhage, cutaneous bleeding, etc. | '''Congenital Afibrinogenemia''' – a genetic disorder that results in the lack of fibrinogen which causes abnormal bleeding including gastrointestinal hemorrhage, cutaneous bleeding, etc. | ||
'''Hepatic fibrinogen storage disease''' - occurs when there is a mutation in the γ chain which causes the storage of fibrinogen in the ER of liver cells. The storage of fibrinogen in these cells can cause liver disease. | '''Hepatic fibrinogen storage disease''' - occurs when there is a mutation in the γ chain which causes the storage of fibrinogen in the ER of liver cells. The storage of fibrinogen in these cells can cause liver disease. | ||
'''Aquired Dysfibrinogenemia''' - may occur as a result of liver disease which causes an error during fibrinogen synthesis resulting in dysfunctional fibrinogen | '''Aquired Dysfibrinogenemia''' - may occur as a result of liver disease which causes an error during fibrinogen synthesis resulting in dysfunctional fibrinogen | ||