, deaths were caused by delays in receiving effective emergency treatment and/or not receiving effective drug therapy, i.e., C1INH
concentrate, ecallantide, or icatibant.
Inhibitor proteins regulate complement activation, including C1 inhibitor (C1INH
), carboxypeptidase N (CPN; which inactivates the anaphylatoxins C3a, C4a, and C5a), and factor I (which inactivates C3b and C4b, using C4bbinding protein (C4BP)).
Patients have low levels or activity of C1 esterase inhibitor (C1INH), C4, and C1q.
In HAE type I, a gene mutation results in decreased plasma antigenic and functional levels of C1INH, which affect 85% of patients.
Rhucin (INN conestat alfa) is a recombinant version of the human protein C1 inhibitor (C1INH
Ruconest is a recombinant version of the human C1 inhibitor (C1INH) protein approved for the treatment of angioedema attacks in patients with HAE in all 27 EU countries plus Norway, Iceland and Liechtenstein.
The study was an open-label exploratory Phase II study of the safety and prophylactic effect of a weekly 50 U/kg rhC1INH treatment in asymptomatic patients with hereditary C1INH deficiency.
Rhucin, recombinant human C1 inhibitor (rhC1INH), is a protein, having identical amino-acid sequence as endogenous human C1INH
. The safety and efficacy of the drug has been demonstrated in two placebo-controlled and four open-label studies.
Under standard SSCP conditions (no PEG additive), DNA amplicons from individuals carrying the Val 458/Met 458 polymorphism in exon 8 of the C1 inhibitor gene (C1INH
)  could not be separated into single-strand conformers; however, these conformers clearly migrate differently when 5 g/L PEG is added to the gel (Fig.
The authors conclude that rhC1INH constitutes a highly effective alternative to plasma derived C1INH
for the treatment of acute angioedema attacks in HAE patients, the companies said.