Role of calcium in blood clotting schematic re
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Calcium mediates the binding of the complexes via the terminal gamma-carboxy residues on FXa and FIXa to the phospholipid surfaces expressed by platelets, as well as procoagulant microparticles or microvesicles shed from them. Calcium is also required at other points in the coagulation cascade.
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Number and/or name Function Associated genetic disorders
I (fibrinogen) Forms clot (fibrin) Congenital afibrinogenemia, Familial renal amyloidosis
II (prothrombin) Its active form (IIa) activates I, V, VII, VIII, XI, XIII, protein C, platelets Prothrombin G20210A, Thrombophilia
III (tissue factor or tissue thromboplastin) Co-factor of VIIa (formerly known as factor III)
IV (calcium) Required for coagulation factors to bind to phospholipid (formerly known as factor IV)
V (proaccelerin, labile factor) Co-factor of X with which it forms the prothrombinase complex Activated protein C resistance
VI Unassigned – old name of Factor Va
VII (stable factor, proconvertin) Activates IX, X congenital factor VII deficiency
VIII (Antihemophilic factor A) Co-factor of IX with which it forms the tenase complex Haemophilia A
IX (Antihemophilic factor B or Christmas factor) Activates X: forms tenase complex with factor VIII Haemophilia B
X (Stuart-Prower factor) Activates II: forms prothrombinase complex with factor V Congenital Factor X deficiency
XI (plasma thromboplastin antecedent) Activates IX Haemophilia C
XII (Hageman factor) Activates factor XI, VII, prekallikrein and plasminogen Hereditary angioedema type III
XIII (fibrin-stabilizing factor) Crosslinks fibrin Congenital Factor XIIIa/b deficiency
von Willebrand factor Binds to VIII, mediates platelet adhesion von Willebrand disease
prekallikrein (Fletcher factor) Activates XII and prekallikrein; cleaves HMWK Prekallikrein/Fletcher Factor deficiency
high-molecular-weight kininogen (HMWK) (Fitzgerald factor) Supports reciprocal activation of XII, XI, and prekallikrein Kininogen deficiency
fibronectin Mediates cell adhesion Glomerulopathy with fibronectin deposits
antithrombin III Inhibits IIa, Xa, and other proteases Antithrombin III deficiency
heparin cofactor II Inhibits IIa, cofactor for heparin and dermatan sulfate ("minor antithrombin") Heparin cofactor II deficiency
protein C Inactivates 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
I (fibrinogen) Forms clot (fibrin) Congenital afibrinogenemia, Familial renal amyloidosis
II (prothrombin) Its active form (IIa) activates I, V, VII, VIII, XI, XIII, protein C, platelets Prothrombin G20210A, Thrombophilia
III (tissue factor or tissue thromboplastin) Co-factor of VIIa (formerly known as factor III)
IV (calcium) Required for coagulation factors to bind to phospholipid (formerly known as factor IV)
V (proaccelerin, labile factor) Co-factor of X with which it forms the prothrombinase complex Activated protein C resistance
VI Unassigned – old name of Factor Va
VII (stable factor, proconvertin) Activates IX, X congenital factor VII deficiency
VIII (Antihemophilic factor A) Co-factor of IX with which it forms the tenase complex Haemophilia A
IX (Antihemophilic factor B or Christmas factor) Activates X: forms tenase complex with factor VIII Haemophilia B
X (Stuart-Prower factor) Activates II: forms prothrombinase complex with factor V Congenital Factor X deficiency
XI (plasma thromboplastin antecedent) Activates IX Haemophilia C
XII (Hageman factor) Activates factor XI, VII, prekallikrein and plasminogen Hereditary angioedema type III
XIII (fibrin-stabilizing factor) Crosslinks fibrin Congenital Factor XIIIa/b deficiency
von Willebrand factor Binds to VIII, mediates platelet adhesion von Willebrand disease
prekallikrein (Fletcher factor) Activates XII and prekallikrein; cleaves HMWK Prekallikrein/Fletcher Factor deficiency
high-molecular-weight kininogen (HMWK) (Fitzgerald factor) Supports reciprocal activation of XII, XI, and prekallikrein Kininogen deficiency
fibronectin Mediates cell adhesion Glomerulopathy with fibronectin deposits
antithrombin III Inhibits IIa, Xa, and other proteases Antithrombin III deficiency
heparin cofactor II Inhibits IIa, cofactor for heparin and dermatan sulfate ("minor antithrombin") Heparin cofactor II deficiency
protein C Inactivates 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
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