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Coagulation
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==List of coagulation factors== There are 13 traditional clotting factors, as named below,<ref name="Openstax Anatomy & Physiology attribution">{{CC-notice|cc=by4|url=https://openstax.org/books/anatomy-and-physiology/pages/18-5-hemostasis}} {{Cite book |last=Betts |first=J Gordon |title=Anatomy & Physiology |last2=Desaix |first2=Peter |last3=Johnson |first3=Eddie |last4=Johnson |first4=Jody E |last5=Korol |first5=Oksana |last6=Kruse |first6=Dean |last7=Poe |first7=Brandon |last8=Wise |first8=James |last9=Womble |first9=Mark D |date=2023 |publisher=OpenStax CNX |isbn=978-1-947172-04-3 |location=Houston |chapter=Β§18.5 Homeostasis |chapter-url=https://openstax.org/books/anatomy-and-physiology/pages/18-5-hemostasis |last10=Young |first10=Kelly A}}</ref> and other substances necessary for coagulation: {| class="wikitable" |+ Coagulation factors and related substances |- ! Number/Name !! Synonym(s) !! Function !! Associated genetic disorders !! Type of molecule !! Source !! Pathway(s) |- | Factor I || [[Fibrinogen]] || Forms fibrin threads in blood clots || {{blist|[[Congenital afibrinogenemia]]|[[Familial renal amyloidosis]]}} || [[Plasma protein]] || Liver || Common pathway; converted into fibrin |- | Factor II* || [[Prothrombin]] || Its active form (IIa) activates [[platelet]]s, factors I, V, VII, VIII, XI, XIII, [[protein C]] || {{blist|[[Thrombophilia]]|[[Prothrombin thrombophilia]] (Prothrombin G20210A)<ref name="GHR2018">{{Cite web |title=Prothrombin thrombophilia |url=https://medlineplus.gov/genetics/condition/prothrombin-thrombophilia/ |url-status=live |archive-url=https://web.archive.org/web/20230912225514/https://medlineplus.gov/genetics/condition/prothrombin-thrombophilia/ |archive-date=12 September 2023 |access-date=2023-09-11 |website=MedlinePlus}}</ref>}} | Plasma protein || Liver || Common pathway; converted into thrombin |- | Factor III|| {{blist|[[Tissue factor]] |tissue thromboplastin}} || Co-factor of factor VIIa, which was formerly known as factor III || || Lipoprotein mixture || Damaged cells and platelets || Extrinsic |- | Factor IV || {{blist|Calcium|Calcium ions|Ca<sup>2+</sup> ions}} || Required for coagulation factors to bind to phospholipids, which were formerly known as factor IV || || Inorganic ions in plasma || Diet, platelets, bone matrix || Entire process of coagulation |- | [[Factor V]] || {{blist|Proaccelerin|labile factor|Ac-globulin}} || Co-factor of factor X with which it forms the [[prothrombinase]] complex || [[Activated protein C resistance]] || Plasma protein || Liver, platelets || Extrinsic and intrinsic |- | Factor VI || {{blist|''Unassigned'' β<br>old name of factor Va<br>(activated form of factor V)|accelerin (formerly)}} || N/A || N/A || N/A || |- | [[Factor VII]]* || {{blist|Proconvertin|Serum Prothrombin Conversion Accelerator (SPCA)|Stable factor}} || Activates factors IX, X; increases rate of catalytic conversion of prothrombin into thrombin || Congenital [[factor VII deficiency]] || Plasma protein || Liver || Extrinsic |- | [[Factor VIII]] || {{blist|Antihemophilic factor A|Antihemophilic factor (AHF)|Antihemophilic globulin (AHG)}} || Co-factor of factor IX with which it forms the [[tenase]] complex || [[Hemophilia A]] || Plasma protein factor || Platelets and endothelial cells || Intrinsic |- | [[Factor IX]]* || {{blist|Antihemophilic factor B|Christmas factor|plasma thromboplastin component (PTC)}} || Activates factor X, forms [[tenase]] complex with factor VIII || [[Hemophilia B]] || Plasma protein || Liver || Intrinsic |- | [[Factor X]]* || {{blist|Stuart-Prower factor|Stuart factor}} || Activates factor II, forms [[prothrombinase]] complex with factor V || Congenital Factor X deficiency || Protein || Liver || Extrinsic and intrinsic |- | [[Factor XI]] || {{blist|Plasma thromboplastin antecedent (PTA)|Antihemophilic factor C}} || Activates factor IX || [[Hemophilia C]] || Plasma protein || Liver || Intrinsic |- | [[Factor XII]] || Hageman factor || Activates XI, VII, prekallikrein and plasminogen || {{nobr|[[Hereditary angioedema]] type III}} || Plasma protein || Liver || Intrinsic; initiates clotting in vitro; also activates plasmin |- | [[Factor XIII]] || Fibrin-stabilizing factor || Crosslinks fibrin threads || Congenital factor XIIIa/b deficiency || Plasma protein || Liver, platelets || Common pathway; stabilizes fibrin; slows down fibrinolysis |- | [[Vitamin K]] || Clotting vitamin || Essential factor to the hepatic [[gamma-glutamyl carboxylase]] that adds a [[carboxyl]] group to [[glutamic acid]] residues on factors II, VII, IX and X, as well as [[Protein S]], [[Protein C]] and [[Protein Z]]<ref name="Vitamin K">{{Cite book |last=Waller |first=Derek G. |title=Medical Pharmacology and Therapeutics |last2=Sampson |first2=Anthony P. |date=2018 |isbn=978-0-7020-7167-6 |pages=175β90 |chapter=Haemostasis |doi=10.1016/B978-0-7020-7167-6.00011-7}}</ref> || [[Vitamin K deficiency]] || Phytyl-substituted naphthoquinone derivative || [[Gut microbiota]]<br>(e.g. [[Escherichia coli|E. coli]]<ref>{{Cite journal |last=Blount |first=Zachary D. |date=2015-03-25 |title=The unexhausted potential of E. coli |journal=eLife |volume=4 |page=e05826 |doi=10.7554/eLife.05826 |pmc=4373459 |pmid=25807083 |doi-access=free}}</ref>),<br>dietary sources || Extrinsic<ref>{{Cite web |title=Coagulation Cascade: What Is It, Steps, and More {{!}} Osmosis |url=https://www.osmosis.org/answers/coagulation-cascade#:~:text=The%20extrinsic%20pathway%20begins%20when,vitamin%20K%20to%20be%20activated. |url-status=live |archive-url=https://web.archive.org/web/20230908085238/https://www.osmosis.org/answers/coagulation-cascade#:~:text=The%20extrinsic%20pathway%20begins%20when,vitamin%20K%20to%20be%20activated. |archive-date=2023-09-08 |access-date=2023-09-08 |website=www.osmosis.org}}</ref> |- | [[von Willebrand factor]] || || Binds to VIII, mediates platelet adhesion || [[von Willebrand disease]] || Blood glycoprotein || Blood vessels' [[endothelia]],<br> bone marrow<ref>{{Cite web |title=VWF gene: MedlinePlus Genetics |url=https://medlineplus.gov/genetics/gene/vwf/#:~:text=Von%20Willebrand%20factor%20is%20made,by%20an%20enzyme%20called%20ADAMTS13. |url-status=live |archive-url=https://web.archive.org/web/20230511200104/https://medlineplus.gov/genetics/gene/vwf/#:~:text=Von%20Willebrand%20factor%20is%20made,by%20an%20enzyme%20called%20ADAMTS13. |archive-date=11 May 2023 |access-date=2023-09-08 |website=medlineplus.gov}}</ref> || |- | [[Prekallikrein]] || Fletcher factor || Activates XII and prekallikrein; cleaves HMWK || Prekallikrein/Fletcher factor deficiency |- | [[Kallikrein]] || || Activates plasminogen || |- | [[High-molecular-weight kininogen]] || {{blist|Fitzgerald factor|HMWK}} || Supports reciprocal activation of factors XII, XI, and prekallikrein || [[Kininogen deficiency]] |- | [[Fibronectin]] || || Mediates cell adhesion || [[Glomerulopathy]] with fibronectin deposits |- | [[Antithrombin]] III || || Inhibits factors IIa, Xa, IXa, XIa, and XIIa || [[Antithrombin III deficiency]] |- | [[Heparin cofactor II]] || || Inhibits factor IIa, cofactor for heparin and [[dermatan sulfate]] ("minor antithrombin") || Heparin cofactor II deficiency |- | [[Protein C]] || || Inactivates factors Va and VIIIa || [[Protein C deficiency]] |- | [[Protein S]] || || Cofactor for activated protein C (APC, inactive when bound to C4b-binding protein || [[Protein S deficiency]] |- | [[Protein Z]] || || Mediates thrombin adhesion to phospholipids and stimulates degradation of factor X by ZPI || [[Protein Z deficiency]] |- | [[Protein Z-related protease inhibitor]] || ZPI || Degrades factors X (in presence of protein Z) and XI (independently || |- | [[Plasminogen]] || || Converts to plasmin, lyses fibrin and other proteins || Plasminogen deficiency type I (ligneous conjunctivitis) |- | [[alpha 2-antiplasmin|Ξ±<sub>2</sub>-Antiplasmin]] || || Inhibits plasmin || Antiplasmin deficiency |- | [[alpha-2-Macroglobulin|Ξ±<sub>2</sub>-Macroglobulin]] || || Inhibits plasmin, kallikrein, and thrombin || |- | [[Tissue plasminogen activator]] || t-PA or TPA || Activates plasminogen || {{blist|Familial [[hyperfibrinolysis]]|[[Thrombophilia]]}} |- | [[Urokinase]] || || Activates plasminogen || [[Quebec platelet disorder]] |- | [[Plasminogen activator inhibitor-1]] || PAI-1 || Inactivates tPA and urokinase (endothelial PAI || Plasminogen activator inhibitor-1 deficiency |- | [[Plasminogen activator inhibitor-2]] || PAI-2 || Inactivates tPA and urokinase || Plasminogen activator inhibitor-1 deficiency |- | [[Cancer procoagulant]] || || Pathological activator of [[factor X]]; linked to thrombosis in various [[cancer]]s<ref>{{Cite journal |last=Gordon |first=S. G. |last2=Mielicki |first2=W. P. |date=March 1997 |title=Cancer procoagulant: a factor X activator, tumor marker and growth factor from malignant tissue |journal=Blood Coagulation & Fibrinolysis |volume=8 |issue=2 |pages=73β86 |doi=10.1097/00001721-199703000-00001 |issn=0957-5235 |pmid=9518049}}</ref>|| |- ! colspan=7 | <nowiki>*</nowiki> Vitamin K is required for biosynthesis of these clotting factors<ref name="Vitamin K" /> |}
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