Sandbox GGC14: Difference between revisions
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== Structure == | == Structure == | ||
Fibrinogen is located in the circulatory system as part of plasma, it is readily available | |||
Fibrinogen is a glycoprotein made up of two subunits which include <scene name='78/781216/Abysubunitsrbg/5'>three non-identical chains</scene> Aα, Bβ γ. Its central region also called “E region” is where all chains meet, the chains intertwine with each other to hold both of the subunits together. At the ends of the a and b chains are fibrinopeptides, fibrinopeptide A is about 16 amino acids long, while fibrinopeptide B is around 15 amino acids long. These small peptides become very important when activating fibrinogen. On both ends of fibrinogen are the D regions containing the β & γ nodule and the coiled-coil region. | Fibrinogen is a glycoprotein made up of two subunits which include <scene name='78/781216/Abysubunitsrbg/5'>three non-identical chains</scene> Aα, Bβ γ. Its central region also called “E region” is where all chains meet, the chains intertwine with each other to hold both of the subunits together. At the ends of the a and b chains are fibrinopeptides, fibrinopeptide A is about 16 amino acids long, while fibrinopeptide B is around 15 amino acids long. These small peptides become very important when activating fibrinogen. On both ends of fibrinogen are the D regions containing the β & γ nodule and the coiled-coil region. There are carbohydrates throughout the structure | ||
== Function == | == Function == | ||
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αγβ | αγβ | ||
== Disease == | == Disease == | ||
'''Congenital Afibrinogenemia''' – a genetic disorder that results in the lack of fibrinogen which causes abnormal bleeding | '''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. | |||
'''Aquired Dysfibrinogenemia''' - may occur as a result of liver disease which causes an error during fibrinogen synthesis resulting in dysfunctional fibrinogen | |||
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1. Acharya, S. S., & Dimichele, D. M. (2008). Rare inherited disorders of fibrinogen. Haemophilia, 14(6), 1151–1158. doi: 10.1111/j.1365-2516.2008.01831.x | 1. Acharya, S. S., & Dimichele, D. M. (2008). Rare inherited disorders of fibrinogen. Haemophilia, 14(6), 1151–1158. doi: 10.1111/j.1365-2516.2008.01831.x | ||
2. Doolittle, R., Kollman, J., Sawaya, M., Pandi, L., & Riley, M. (2009). Crystal Structure of Human Fibrinogen. American Chemical Society. doi: 10.2210/pdb3ghg/pdb | 2. Al-Hussaini, A., Altalhi, A., Hag, I. E., Alhussaini, H., Francalanci, P., Giovannoni, I., & Callea, F. (2014). Hepatic fibrinogen storage disease due to the fibrinogen γ375 Arg → Trp mutation "fibrinogen aguadilla" is present in Arabs. Saudi Journal of Gastroenterology, 20(4), 255. doi: 10.4103/1319-3767.136985 | ||
3. Doolittle, R., Kollman, J., Sawaya, M., Pandi, L., & Riley, M. (2009). Crystal Structure of Human Fibrinogen. American Chemical Society. doi: 10.2210/pdb3ghg/pdb | |||
4. Köhler, S., Schmid, F., & Settanni, G. (2015). The Internal Dynamics of Fibrinogen and Its Implications for Coagulation and Adsorption. PLOS Computational Biology, 11(9). doi: 10.1371/journal.pcbi.1004346 | |||
5. Medved, L., & Weisel, J. W. (2009). Recommendations for nomenclature on fibrinogen and fibrin. Journal of Thrombosis and Haemostasis, 7(2), 355–359. doi: 10.1111/j.1538-7836.2008.03242.x | |||