H ereditary angio-oedema (HAE; OMIM#106100) affects 1 in 50 000 of the population and results from deficiency of the plasma protein C1 inhibitor. It is characterised clinically by recurrent episodes of subcutaneous, intestinal, and laryngeal oedema, which vary in severity between affected individuals. In some cases the laryngeal oedema may be so severe as to occlude the upper airway and threate...

caused by autoantibodies against C1-INH (AAE type II). Reduced C1-INH activity leads to uncontrolled bradykinin formation and to angioedema symptoms. There is no established treatment for AAE. Replacement with plasma-derived C1-INH is effective in most patients with life-threatening attack; however, icatibant, a bradykinin B2 receptor antagonist, may represent an alternative treatment. Methods:...

Purified preparations of normal Cl-inhibitor (Ci-INH) formed high mol wt complexes with plasma kallikrein that were stable during sodium dodecyl sulfate (SDS)-gel electrophoresis, but most of the dysfunctional Cl -INH proteins isolated from plasma of patients with type II hereditary angioneurotic edema (HANE) did not. Two of eight dysfunctional Cl -INH proteins were cleaved to lower mol wt form...

In migraine and other primary headaches there is a strong vascular component. Besides the trigeminovascular components some of the associated symptoms point to the involvement of brain stem regions. The central limb of the trigeminal vascular pathway is its projection to the trigeminal nucleus caudalis (TNC) and to the C1-C2 levels of the spinal cord. The aim of the present study was to demonst...

C1 inhibitor is a heavily glycosylated plasma protein that regulates the activity of the first component of complement (Cl) by inactivation of the serine protease subcomponents, COr and Cls. C1 inhibitor cDNA clones have been isolated, and one of these (pClINH1, 950 base pairs) has been partially sequenced. Sequence analysis demonstrates that the C1 inhibitor is a member of the serpin "superfam...

Several types of angioedema exist beyond hereditary angioedema (HAE) types I/II; however, the diagnostic and treatment needs of these conditions are not well understood. Noticeably, there are no licensed treatments available for other forms of angioedema beyond HAE types I/II, and similarly they are unresponsive to conventional antihistamine/glucocorticoid treatment. A group of angioedema exper...

Study Design A systematic review of the literature. Objectives To review the published results to date of motion-preserving direct reconstruction of C1 ring fractures with combined coronal plane displacement of at least 7 mm (rule of Spence) and so at risk for Dickman type I or II disruption of the transverse atlantal ligament (TAL). Methods A structured literature review prompted by successful...

We study the tachyon condensation of the DD̄-brane system with a constant tachyon vev in the context of classical solutions of the Type II supergravity. We find that the general solution with the symmetry ISO(1, p)×SO(9−p) (the three-parameter solution) includes the extremal black p-brane solution as an appropriate limit of the solution with fixing one of the three parameters (c1). Furthermore, ...