Blood Flashcards
Which factor is deficient in Haemophilia B?
Factor IX (9)
Which factor is deficient in haemophilia C?
Factor XI (10)
What is the genetic inheritance of Haemophilia A?
X-linked recessive
Which factor is deficient in Hameophilia A
Factor VIII (8)
What is the co-factor needed for the Carboxylase enzyme?
Vitamin K
What is the role of the Carboxylase enzyme, with regards to blood funciton? Why is it essential in blood clotting?
Carboxylation of the Gla domains of prothrombin.
Carboxylation gives the prothrombin a negative charge allowing it to bind to the positive Calcium ions (bound to negative platelets)
Why is it important that prothrombin is negatively charged in day to day blood function (IE not clotting)
To prevent the prothrombin binding to the negatively charged platelets triggering the formation of a clot. (They repellent each other due to their -ve charge)
Where is prothrombin carboxylated?
In the ER of liver cells
What cleaves the -vely charged parts on fibrinogen molecules?
Activated thrombin
Once fibrinogen is converted to fibrin, what happens?
(2 things
0
The fibrin molecules aggregate and come together and bind to form a fibrin mesh.
Covalent bonds form between the strands of fibrin
What activates the formation of covalent bonds between strands of fibrin?
Factor XIII
Factor….is fibrin
I
One
Factor… Is thrombin
II
Two
Which factors does thrombin activate in the positive feedback?
5, 7, 8, 11, 13
What does anti-thrombin do in its role in negative feedback?
Reduce the amount of factor X that becomes activated
Reduce the amount of prothrombin cleaved to thrombin