Press release Your Contact News Release Gangolf Schrimpf +49 6151 72-9591 Investor Relations +49 6151 72-3321 October 1, 2015 Merck Agrees to Return Kuvan® Rights to BioMarin Pharmaceutical to Strengthen Focus on Core Business Agreement with BioMarin, a leading company in the treatment of genetic and rare diseases, also includes returning option to develop and commercialize Peg-Pal Merck to receive upfront payment of € 340 million, plus up to € 185 million in additional milestones Darmstadt, Germany, October 1, 2015 - Merck, a leading company for innovative and top-quality high-tech products in healthcare, life science and performance materials, today announced that it has reached an agreement with BioMarin Pharmaceutical, Inc., San Rafael, California, U.S., to return the rights to Kuvan®, used to treat phenylketonuria (PKU), a rare metabolism disorder, as the company focuses its healthcare business on core areas. In addition to Kuvan®, the two companies agreed that Merck will return its option to develop and commercialize Peg-Pal, an investigational drug that is also designed for the treatment of PKU, an autosomal recessive genetic disorder caused by either a defect or a deficiency of the enzyme phenylalanine hydroxylase or its co-factor tetrahydrobiopterin. Merck will receive an upfront payment of € 340 million, equal to five times its annual sales, for Kuvan®, plus up to € 185 million in additional milestones for both products. The agreement is expected to become effective Jan. 1, 2016. 'Returning the rights of Kuvan® and Peg-Pal to BioMarin will allow Merck to fully focus on its core businesses, as well as further align R&D investment behind key strategic areas,' said Belén Garijo, Member of the Executive Board of Merck and CEO Healthcare. Merck KGaA Frankfurter Strasse 250 Head Media Relations -62445 64293 Darmstadt Spokesperson: -9591 / -7144 / -6328 Hotline +49 6151 72-5000 Fax +49 6151 72-3138 Page 1 of 3 'Patients suffering from PKU will continue to benefit from these therapeutic options, as well as from BioMarin's long-term expertise in rare diseases.' Merck remains highly committed to the patients in the field of endocrinology, and in particular to advancing the treatment of growth hormone deficient patients with Saizen®. Over the past years, Merck has re-aligned its healthcare business with a special focus on developing novel therapies in the areas of neurology, oncology, immuno-oncology and... More