نتایج جستجو برای: combined factor v viii deficiency
تعداد نتایج: 1585920 فیلتر نتایج به سال:
Mutations in LMAN1 (ERGIC-53) or MCFD2 cause combined deficiency of factor V and factor VIII (F5F8D). LMAN1 and MCFD2 form a protein complex that functions as a cargo receptor ferrying FV and FVIII from the endoplasmic reticulum to the Golgi. In this study, we analyzed 10 previously reported and 10 new F5F8D families. Mutations in the LMAN1 or MCFD2 genes accounted for 15 of these families, inc...
BACKGROUND: Elevated plasma levels of coagulation factor VIII are a strong risk factor for pulmonary emboli and deep venous thromboses. OBJECTIVES: To identify reversible biomarkers associated with high factor VIII, and assess potential significance in a specific at-risk population. PATIENTS/METHODS: 609 patients with hereditary haemorrhagic telangiectasia were recruited prospectively in two se...
BACKGROUND AND OBJECTIVES Factor V HR2 possesses decreased co-factor activity to activated protein C and an increased ratio of factor V1 to factor V2. Factor V HR2 is associated with a mild increase in the risk of venous thromboembolism although not all studies concur on this point. DESIGN AND METHODS Inconsistencies in results of the epidemiological studies may stem from a failure to identif...
recB and/or recC deficiency in Escherichia coli K-12 is indirectly suppressed by the presence of sbcA(-) mutations. sbcA(-) strains contain an increased level of an ATP-independent nuclease. Genetic and enzymatic tests indicate that this activity is not exonuclease III, exonuclease V (recB-recC nuclease), DNA polymerase I, or lambda exonuclease. This new enzyme (exonuclease VIII) has been purif...
We prepared a highly purified and relatively heat stable form of factor VIII which contained 25 units per mL (u/mL) activity using PEG-4000 and developed an effective and new manufacturing process. Heat treatment was performed at 80°C for 72 hrs in the presence of different stabilizers. In our studies, we used different organic solvents as preservatives to maintain factor VIII activity, sin...
Normal human plasma contains a complex of two proteins that are important in hemostasis and coagulation. The factor VIII procoagulant protein (antihemophilic factor) and the factor VIll-related protein (von Willebrand factor) are under separate genetic control, have distinct biochemical and immunologic properties, and have unique and essential T HE IMPORTANCE of factor VIII in hemostasis and bl...
objective(s): the purpose of the present study was the immunohistochemical evaluation of vegf and vii factors in dog’s teeth pulp revascularized with mta and propolis. materials and methods: 144 mature and immature two rooted dog’s premolar canals were selected. pulp necrosis and infection were established after 2 weeks and the disinfection of the canals was done with copious naocl irrigation ...
Most plasmas from patients with inhibitors contain antibodies that are reactive with the C2 domain of factor VIII. Previously, we have shown that the variable heavy chain (V(H)) regions of antibodies to the C2 domain are encoded by the closely related germline gene segments DP-10, DP-14, and DP-88, which all belong to the V(H)1 gene family. Here, we report on the isolation and characterization ...
Plasma protein C exerts anticoagulatory effects by inactivating factors V and VIII. Hereditary protein C deficiency is transmitted as an autosomal dominant disorder. Homozygous individuals usually develop purpura fulminans as newborns; heterozygous protein C-deficient individuals are at increased risk for venous thrombosis and pulmonary embolism. However, arterial thrombosis has been only rarel...
نمودار تعداد نتایج جستجو در هر سال
با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید