Unfolded proteins in the endoplasmic reticulum cause trans-autophosphorylation of the bifunctional transmembrane kinase Ire1, which induces its endoribonuclease activity. The endoribonuclease initiates nonconventional splicing of HAC1 messenger RNA to trigger the unfolded-protein response (UPR). We explored the role of Ire1's kinase domain by sensitizing it through site-directed mutagenesis to ...

factor v (fv) deficiency is a rare bleeding disorder (rbd) that inherit in autosomal recessive manner. diagnosis of fv deficiency (fvd) is made by routine coagulation tests, fv activity and molecular analysis. in patients with fvd, routine coagulation tests including activated partial thromboplastin time (aptt), prothrombin time (pt) and evenbleeding time (bt) are prolongedwhile thrombin time (...

Recently we have described a normal plasma activity that modulates contact activation by inhibiting adsorption of factor XI to activating surfaces. Here we report the first identified case in which a patient has abnormal clotting tests due to an excess of a similar activity. The patient's plasma had a prolonged partial thromboplastin time and low apparent factor XI assay. His plasma prolonged t...

purpose: due to unhealthy lifestyles, there has been an increase in the prevalence of coronary artery disease (cad) and a reduction in its age of onset. given the high cost of diagnosis and treatment of cad, there is an urgent need to teach people strategies helpful in changing their lifestyles as this can help reduce the risk factors of the disease. therefore, the goal of the present study was...

Measurement of serum angiotensin-converting enzyme (ACE) is extremely useful as an aid in the diagnosis and longitudinal evaluation of patients with sarcoidosis. We have detected a human serum ACE-inhibitor which affects the ACE level obtained by activity measurements. The effect of the inhibitor can be eliminated by a mere eight-fold dilution of the serum sample with physiologic saline. We rec...

A coagulation Factor V inhibitor developed in a man 75 yr of age in association with an anaplastic malignancy and drug treatment (including the aminoglycoside antibiotic, gentamicin). The patient did not bleed abnormally, despite both surgical challenge and plasma Factor V activity of less than 1%. The inhibited plasma had grossly prolonged prothrombin and activated partial thromboplastin times...

A 42-yr-old woman with systemic lupus erythematosus without bleeding diathesis developed a prolonged activated partial thromboplastin time that was not corrected by normal plasma. An inhibitor that acted rapidly and inactivated 0.5 U/ml plasma thromboplastin antecedent (PTA, factor XI) at a 1:200 plasma dilution was demonstrated. In addition to a low titer of PTA (less than 0.01 U/ml), plasma a...