1. Page 1 Corporate Overview 2012 Dr. Andreas Diedenhofen

2. Page 2 Dr. Claudio Cavazza Founded the Sigma-Tau Group 1957  4 May 1934, Bologna  6 June 2011, Rome L-Carnitine The favourite product of Dr. Cavazza More than 2,000 patents Countless research projects

3. Page 3 Privately owned, R&D driven pharmaceutical company Sales & Marketing of pharmaceuticals and nutritional supplements 9th largest pharmaceutical group in Italy in 2010 Presence in the USA and Asia (India and China) Key target are orphan indications R&D efforts are focused on: - Carnitine system - Oncology and autoimmune diseases - Rare diseases - Malaria sigma-tau Group

4. Page 4 sigma-tau Group Milestones 1957-sigma-tau S.p.A. founded by Emilio and Claudio Cavazza. Owned by the Cavazza family 1979-Incorporation of Biosint S.p.A. (Sermoneta) - Production site for carnitine, its salts and derivatives) -Incorporation of Avantgarde S.p.A. (Pomezia) -OTC products, personal care/cosmetic products 1985-Incorporation of Prassis Institute S.p.A. (Milan) - R&D center specializing cardiovascular diseases 1986-sigma-tau Fondazione (Rome) - Promotion of culture and science 1987-Acquisition of Tecnogen S.p.A. - Biotech R&D company (monoclonal antibodies and recombinant proteins) 1998-sigma-tau HealthScience S.r.l. - Development of nutritional supplements. 1999-Consolidation of commercial affiliates in USA and Europe (France, Spain, The Netherlands, Germany, Switzerland and Portugal)

5. Page 5 sigma-tau Group Milestones 2001- Expansion in the Italian Market creating Biofutura Pharma S.p.A. (Milan) 2005- Creation of a new EU affiliate: sigma-tau Pharma Ltd. in UK 2007- Incorporation of sigma-tau Research Switzerland s.a. located in Mendrisio (CH) - Consolidation in sigma-tau Group (28.4%) of Lee’s Pharmaceutical Holdings Ltd. based in Hong Kong and covering the Chinese market, sigma-tau being the major shareholder 2008- Incorporation of sigma-tau India Private Ltd. 2009- (July) Submission/filing of Eurartesim to the EMA - Incorporation of sigma-tau Pharma Belgium SPRL located in Etterbeek - Brussels 2010 - (January) Acquisition of Enzon’s “specialty care” business - Integration of Enzon’s manufacturing facility as sigma-tau PharmaSource Inc. 2011- EMA approval of Eurartesim

6. Page 6 sigma-tau Group Geographic Overview sigma-tau S.p.A. Avantgarde S.p.A. Tecnogen S.p.A. sigma-tau Generics S.r.l. Biosint S.p.A. Prassis S.p.A. - Rostaquo S.p.A. Biofutura Pharma S.p.A sigma-tau India Private Ltd. (Mumbai) sigma-tau B.V. sigma-tau HS Int. B.V. sigma-tau Pharma Ltd. sigma-tau GmbH sigma-tau Pharma A.G. sigma-tau Research Switzerland SA sigma-tau S.a.r.l. Defiante Farmaceutica Lda sigma-tau España S.A. sigma-tau Pharma Belgium SPRL sigma-tau Pharmaceuticals, Inc. (Gaithersburg - MD) sigma-tau Healthscience, LLC. (Gaithersburg - MD) sigma-tau PharmaSource, Inc. (Indianapolis) Lee’s Pharmaceutical Holdings Ltd. (Hong Kong)

7. Page 7 sigma-tau Group Sales 2010 By business area (€ mio) Total sales: 664,39 € mio Pharma Rare Disease Nutraceutical Personal Care Chemical 491,92102,05 36,03 22,02 12,37

8. Page 8 Italy sigma-tau Group Commercial Partnerships

9. Page 9 sigma-tau Group Commercial Activities

10. Page 10 sigma-tau S.p.A. Location Pomezia (Rome) Annual Sales 2010 270 € mio (*) Employees 1,566 Medical Representatives547 (Italy only) Main Production Plant Research Centre (R&D) * Products distributed by Avantgarde excluded Last updated Dec. 2010

11. Page 11 RESPIRATORY Eolus® Zinnat® Zaditen ® Tau-Tux® CNS Anafranil® Trizadol ® Depamag® Hydergina® CARNITINES Nicetile® Carnitene® Dromos® OTHERS Bentelan® Debridat® Debrum® Indoxen® Synachten® Syntocinon® Eufans® Tript-oh® Zadaxin® Annual Pharma sales (2010): 90,36 € mio of which Food supplements & Medical devices products: 11,81 € mio Medical Representatives: 158 reps Biofutura Pharma S.p.A. 151 Medical Reps GP Line7 Medical Reps Specialist Line 15 District Managers FOOD SUPPLEMENTS Carnidyn® Carnidyn Plus® Resvis XR Biofutura® Resvis Forte XRBiofutura® Amedial BF® Flebinec® MEDICAL DEVICES Condrotide®

12. Page 12 Consumer products (pharmacy only) Skin care Oral care Pharmaceutical products OTC Rx Food supplements Medical devices Annual sales (2010) 95,75 € mio Medical Reps: - 48 pharmacy Reps (*) - 39 Reps 3,000 Dermatologists 5,500 Gynecologists 3,000 Paediatricians Avantgarde S.p.A. 9,500 Pharmacies Targets (Italy) Pharmacies - 17,700 Dermatologists - 3,500 Gynecologists - 9,000 Paediatricians - 10,000 (*) Distribution of off-patent branded products Medical Reps report functionally to Avantgarde’s S.p.A. Scientific Service

13. Page 13 sigma-tau España S.A. Location: Madrid Annual sales (2010): 17,80 € mio Field force: 59 retail reps sigma-tau in Europe sigma-tau S.a.r.L. Location: Paris Annual sales (2010): 29,57 € mio Field force: 17 hospital reps - 70/140 retail agents sigma-tau AG Location: Zofingen (Zurich) Annual sales (2010): 7,84 € mio Field force: 10 sigma-tau BV / sigma-tau HealthScience Int. BV Location: Utrecht (Holland) Annual sales (2010): 8,10 € mio Marketing: 4 sigma-tau Pharma Belgium Sprl Location: Etterbeek (Brussels) Annual Sales (2010): 2,14 € mio sigma-tau GmbH Location: Düsseldorf Annual sales (2010): 4,64 € mio Marketing: 5 Defiante Pharmaceutica Ltd. Location: Madeira Annual sales (2010): 31,39 € mio (*) Marketing: 2 sigma-tau Pharma Ltd. Location: Reading (London) Annual sales (2009): 1,35 € mio Medical marketing: 1 (*) Only pharm. sales vs. third parties

14. Page 14 sigma-tau Pharmaceuticals Inc. Location: Maryland Annual sales (2010): 81,81 € mio (2009: 19,7 € mio) Med Reps: 22 www.sigmatau.com Sigma-Tau Pharmaceuticals, Inc. is dedicated to developing and providing innovative therapies to patients who suffer from rare diseases. Product portfolio Oncaspar ® pegasparaginase - an important component in the treatment of acute lymphoblastic leukemia (ALL) Adagen ® pegademase - enzyme replacement therapy for ADA deficiency in patients with severe combined immunodeficiency disease (SCID) Abelcet ® amphotericin B lipid complex – indicated for the treatment of invasive fungal infections Depocyt ® liposomal cytarabine injection - intrathecal treatment of lymphomatous meningitis Carnitor ® L-carnitine - a life-saving treatment for rare metabolic disorders Matulane ® procarbazine - a chemotherapeutic agent used in the treatment of advanced hodgkin’s disease ProXeed Plus ® dietary supplement to enhance male fertility VSL#3 ® medical food used in the management of bowel disorders sigma-tau Group in the US

15. Page 15 Sigma-Tau Group in the US : Rare Diseases Sigma-Tau Pharmaceuticals, Inc is focused on medicines for the unmet needs of patients with rare diseases. Truly unique in its field. Sigma-Tau places its considerable scientific resources behind the discovery of compounds that benefit the few. Simply because it's the right thing to do.

16. Page 16 sigma-tau Group R&D Activities

17. Page 17 Development Projects per Therapeutic Area

18. L-Carnitine to balance L-Carnitine losses due to defects of the so- called carnitine transporter in the cell membrane (substitution in the case of a primary systemic carnitine deficiency) to balance L-Carnitine losses, which may have been caused by various diseases or as a side effect of certain types of treatment (substitution in the case of a secondary systemic carnitine deficiency) L-Carnitine is used for the treatment of carnitine deficiency diseases

19. Page 19 Malaria Distribution World Map

20. Page 20 Eurartesim Active ingredient: piperaquine dihydroartemisinine Eurartesim® is indicated for the treatment of the uncomplicated plasmodium falciparum malaria in adults, children and infants from 6 months upwards and a body weight of at least 5kg EMA approval Oct.2011 Available: June/July 2012 QUINGHAOSU (Artemisia annua) 黄 花 蒿黄 花 蒿

21. Page 21 21 Sigma-Tau Group – Orphan drugs Products in the Sigma-Tau portfolio following the acquisition of Enzon Pharmaceuticals, Inc. (February 2010) –Oncaspar ® (PEG asparaginase): Component of combination treatment in the case of acute lymphoblastic leukaemia (children and adults) and hypersensitivity to native (released) asparaginase. –Adagen ® (PEG adenosine desaminase (bovine): Severe combined immunodeficiency, genetically caused disorder of the metabolic activities in immunocompetent cells, especially T lymphocytes. –DepoCyt ® (cytarabine): Intrathecal (Injection directly into the cerebral fluid) for the treatment of lymphomatous meningitis (spreading of the lymphoma cells within the cerebral fluid). –Abelcet ® (amphotericin B as lipid complex): Invasive mycoses (mycotic infections), which do not respond to / do not tolerate conventional amphotericine B (without lipid complex).

22. Page 22 Oncaspar ® PEG asparaginase What is Oncaspar ® and what is it used for? Oncaspar® is a cytostatic agent, which reduces the L-Asparagine level within the tumour cells, so that the protein synthesis within these cells is disrupted. Oncaspar ® is used as a component of an antineoplastic combination treatment with re- induction for acute lymphoblastic leukaemia (ALL) in childhood and adulthood in patients with established hypersensitivity to „native“ L- Asparaginases

23. Page 23 Xenbilox ® Cerebrotendinous xanthomatosis / Van- Bogaert-Scherer-Epstein syndrome Genetic defect (sterol 27-hydroxylase gene; deficiency of the mitochondrial enzyme sterol 27-hydroxylase) Increased serum level of a cholesterol metabolite (cholestanol) „Fat“ diarrheas Yellow nodules around the tendons Early-stage cataracts Diseases of the nervous system Dementia; epilepsy Osteoporosis and bone fractures