TBPP_coagulation factors

international unit (IU) = activity per amount of 1 ml pooled plasma, factors: I - XIII all proteins (serinproteases / transglutaminase, co-factor, substrate), factor IV: Calcium, conversion of serinprotease zymogen into the active form

300 kD, 2332 AS, glycoprotein, co-factor of tenase-complex (converts with IXa Factor X into Xa), 0.2 mg/L, trace protein = lowest molecular concentration in blood, heavy chain: A1, A2, B, light chain: A3, C1, C2.

key factor of hemostasis: mediates platelet adherence, carrier of FVIII, concentration: 10mg/L, congenital deficiency --> VW disease (VWD), complex protein: dimerization and multimerization, fusion with albumin

Factor II, VII, IX, X, vitamin K-dependent Serineproteases, posttranslational modification: gamma-carboxylation, highly acidic proteins

vitamin K-dependent, 415 AS, 57 kD, 5mg/L plasma, high degree of post-translational modifications (N-/O-glycosylation, Tyr-sulfation, Ser-phosphorylation, Asp-hydroxylation), half-life of 18-34 hours

vitamin K-dependent, 406 AS, 50 Kd protein, 0.5 mg/L plasma, high degree of post-translational modifications (N-/O-glycosylation, asp-hydroxylation), half life of 2-4 hours

X-linked recessive hereditary bleeding disorders, A: deficiency of Factor VIII, B: deficiency of Factor IX, severe form: spontaneous bleedings, joint bleedings, treatment with substitution therapy: plasma or recombinant factor, IV 2-3 times per week, normal life expectancy and improved quality of life

most severe complication in treatment, development of antibodies against substituted factor (10-20 days), high risk of bleeding, acute treatment with bypass products (activated prothrombin factor concentrate, recombinant FVII) or immune tolerance induction: high doses of FVIII.