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Pharming Group N.V.

Drug Names(s): Recombinant human C1 esterase inhibitor, rhC1INH, conestat alfa, Rhucin

Description: Rhucin is a recombinant human C1 inhibitor. C1 inhibitor (C1-inh) is a single-chain glycoprotein that belongs to a family of serine protease inhibitors termed serpins.

C1-inh is the only natural inhibitor of the serine proteases C1r and C1s of the classical activation pathway of complement. Upon binding to C1r or C1s, C1-inh dissociates the multimolecular classical pathway activation complex, preventing C1s-mediated cleavage of C4 and C2 and the further downstream activation of complement.

Inherited C1-inh deficiencies lead to episodic acute s.c. or mucosal swellings, a condition termed hereditary angioedema.

With regard to pancreatitis, acute pancreatitis is associated with the activation of the complement cascade and the contact (kallikrein-kinin) system allowing for the inhibition by C1-inh. This inhibition of the complement system and kallikrenin-kinin pathway elicits anti-inflammatory properties and a reduction in vascular permeability, respectively, expanding the therapeutic potential of C1-inh.

Deal Structure: Revenue splits for this drug are BioMedTracker estimates.

Pharming had a partnershipwith Esteve on the development, marketing and sales of rhC1INH in Spain, Portugal and Greece.

In August 2011, Pharming Group reached a mutual agreement with Esteve to return the rights to market Ruconest in Spain, Portugal, Andorra and Greece. SOBI now takes up the exclusive distribution rights in these countries, extending its territories for Ruconest to all European Union countries, Iceland, Norway and Switzerland.

Pharming and Paul Royalty Fund
On February 3, 2006 Pharming signed a strategic partnering agreement with affiliates of Paul Royalty Fund II, LP. Under the terms of the agreement, Pharming immediately received an upfront payment of US$ 15.0 million for Pharmings development work on Rhucin and the continued development and commercialization of Rhucin products. In addition, Paul Royalty Fund acquired a total number of 1,094,929 shares at 3.80 per share in exchange of...See full deal structure in Biomedtracker

Partners: Swedish Orphan Biovitrum MegaPharm Ltd. Hyupjin Corporation Transmedic Pte Ltd. CytoBioteck S.A.S. Valeant Pharmaceuticals International, Inc.

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