`UNITED STATES PATENT AND TRADEMARK OFFICE
`
`
`
`
`
`
`
`
`
`
`
`BEFORE THE PATENT TRIAL AND APPEAL BOARD
`
`
`
`
`
`
`
`
`
`
`
`LUPIN LTD. AND LUPIN PHARMACEUTICALS INC.,
`
`Petitioner
`
`v.
`
`HORIZON THERAPEUTICS, LLC,
`
`Patent Owner
`
`
`
`
`
`
`
`
`
`
`
`Case IPR 2017-01159
`
`Patent 9,254,278
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`PATENT OWNER’S INITIAL EXHIBIT LIST
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`
`Pursuant to 37 C.F.R. § 42.63 (e), the Patent Owner hereby provides an
`
`initial exhibit list:
`
`Exhibit
`No.
`
`2001
`
`2002
`
`2003
`
`2004
`
`2005
`
`2006
`
`2007
`
`2008
`
`2009
`
`2010
`
`2011
`
`Description
`
`Declaration of Dr. Gregory M. Enns, M.D., Lupin Ltd. et al. v.
`Horizon Therapeutics, Inc., IPR2016-00829, Exhibit 2006, dated
`February 10, 2017.
`
`RESERVED.
`
`Simell, “Lysinuric Protein Intolerance and Other Cationic
`Aminoacidurias,” in The Metabolic & Molecular Bases of Inherited
`Disease, Ch. 192, pp. 4933-4956 (Scriver et al., eds., 8th ed. 2001).
`(“Simell 2001”).
`
`Singh et al., “Nutritional Management of Urea Cycle Disorders,”
`Crit. Care. Clin. 21:S27-35 (2005). (“Singh”).
`
`U.S. Patent No. 9,095,559. (“’559 Patent”).
`
`Declaration of Dr. Gregory M. Enns, M.D.
`
`Curriculum vitae of Dr. Gregory M. Enns, M.D.
`
`Ari Auron, Patrick D. Brophy, “Hyperammonemia in Review:
`Pathophysiology, Diagnosis, and Treatment,” Pediatric
`Nephrology, 27:207-22 (2012). (“Auron”).
`
`Mark L. Batshaw, et al., “Alternative Pathway Therapy for Urea
`Cycle Disorders: Twenty Years Later,” J. Pediatrics, 38:S46-S55
`(2001). (“Batshaw”).
`
`Nancy E. Maestri, et al., “Prospective Treatment of Urea Cycle
`Disorders,” J. of Pediatrics, 119:923-28, no. 6 (1991). (“Maestri”).
`
`Nancy E. Maestri, et al., “Plasma Glutamine Concentration: A
`Guide in the Management of Urea Cycle Disorders,” J. Pediatrics,
`121:259–61, no. 2 (1992). (“Maestri 1992”).
`
`IPR2017-01160
`
`
`
`
`Page 1
`
`
`
`2012
`
`2013
`
`2014
`
`
`2015
`
`2016
`
`2017
`
`2018
`
`2019
`
`2020
`
`2021
`
`2022
`
`U.S. Patent Publication 2012/0022157 A1, filed August 27, 2009,
`published January 26, 2012. (“’157 App”).
`
`Mendel Tuchman & Mark L. Batshaw, “Management of Inherited
`Disorders of Ureagenesis,” The Endocrinologist 12:99–109, no. 2
`(2002). (“Tuchman”).
`
`Guoyao Wu, “Amino Acids: Metabolism, Functions, and
`Nutrition,” Amino Acids 37:1–17 (2009). (“Wu”).
`
`Alexander Broomfield & Stephen Grunewald, “How to use Serum
`Ammonia,” Archives of Disease in Childhood—Education and
`Practice 97:72–77 (2012). (“Broomfield”).
`
`Fumino Endo, et al., “Clinical Manifestations of Inborn Errors of
`the Urea Cycle and Related Metabolic Disorders During
`Childhood,” J. Nutrition 134:1605S–09S (2004). (“Endo”).
`
`Gregory M. Enns, “Nitrogen Sparing Therapy Revisited 2009,”
`Molecular Genetics and Metabolism 100:S65–S71 (2010). (“Enns
`2010”).
`
`Francois Feillet & J. V. Leonard, “Alternative Pathway Therapy for
`Urea Cycle Disorders,” J. Inherited Metabolic Disease, Supplement
`1, 21:101–111 (1998). (“Feillet”).
`
`Johannes Häberle, et al., “Suggested Guidelines for the Diagnosis
`and Management of Urea Cycle Disorders,” Orphanet J. Rare
`Diseases, 7:32, 1–30 (2012). (“Häberle”).
`
`Johannes Häberle, “Clinical Practice: The Management of
`Hyperammonemia,” Eur. J. of Pediatrics 170:21–34 (2011).
`(“Häberle Clinical”).
`
`J.V. Leonard & A. A. M. Morris, “Urea Cycle Disorders,” Seminars
`in Neonatology 7:27–35 (2002). (“Leonard”).
`
`Ann-Kaisa Niemi & Gregory M. Enns, “Sodium Phenylacetate and
`Sodium Benzoate in the Treatment of Neonatal Hyperammonemia,”
`NeoReviews, 7:e486–e95, no. 9 (2006). (“Niemi”).
`
`IPR2017-01159
`
`
`
`Page 2
`
`
`
`2023
`
`2024
`
`2025
`
`2026
`
`2027
`
`2028
`
`2029
`
`2030
`
`2031
`
`2032
`
`Marshall Summar & Mendel Tuchman, “Proceedings of a
`Consensus Conference for the Management of Patients with Urea
`Cycle Disorders,” J. Pediatrics, 138:S6–S10 (2001). (“Summar”).
`
`Saul W. Brusilow & Nancy E. Maestri, “Urea Cycle Disorders:
`Diagnosis, Pathophysiology, and Therapy,” Advances in Pediatrics
`43:127–70 (1996). (“Brusilow 1996”).
`
`Colloquium, “Consensus Statement from a Conference for the
`Management of Patients with Urea Cycle Disorders,” J. Pediatrics,
`Supplement 1, 138:S1–S5 (2001). (“Consensus”).
`
`“Specialties of Genetics,” Am. Board of Medical Genetics and
`Genomics (last accessed Jan. 17, 2017),
`http://abmgg.org/pages/training_specialties.shtml. (“ABMGG”).
`
`“About Us,” Urea Cycle Disorders Consortium (last accessed Jan.
`17, 2017), https://www.rarediseasesnetwork.org/cms/ucdc/About-
`Us.
`
`Gregory M. Enns, et al., “Survival After Treatment with
`Phenylacetate and Benzoate for Urea-Cycle Disorders,” The New
`England Journal of Medicine 356:2282–92 (2007). (“Enns”).
`
`Gregory M. Enns & Tina M. Cowan, “Hyperammonemia,” in Signs
`and Symptoms of Genetic Conditions: A Handbook, ch. 18, 261–279
`(Louanne Hudgins et al., eds., 2014). (“Enns 2014”).
`
`Michael Msall, et al., “Neurologic Outcome in Children with Inborn
`Errors of Urea Synthesis,” The New England Journal of Medicine
`310:1500–1505 (1984). (“Msall”).
`
`B.D. Cheson, et al., “Novel Therapeutic Agents for the Treatment
`of Myelodysplastic Syndromes,” in Seminars in Oncology, 27:560–
`77, no. 5 (John W. Yarbro, et al. eds., 2000). (“Cheson”).
`
`Fernando Scaglia, et al., “Effect of Alternative Pathway Therapy on
`Branched Chain Amino Acid Metabolism in Urea Cycle Disorder
`Patients,” Molecular Genetics and Metabolism, Supplement 1,
`81:S79-S85 (2004). (“Scaglia”).
`
`IPR2017-01159
`
`
`
`Page 3
`
`
`
`2033
`
`2034
`
`2035
`
`2036
`
`2037
`
`2038
`
`2039
`
`2040
`
`2041
`
`2042
`
`2043
`
`Saul W. Brusilow & Arthur L. Horwich, “Urea Cycle Enzymes,” in
`The Online Metabolic and Molecular Bases of Inherited Disease,
`Ch. 85, pp. 1–89 (David Valle et al. eds., 2015). (“Brusilow
`Online”).
`
`Transcript of January 31, 2017 Deposition of Dr. Keith Vaux, Lupin
`Ltd. et al. v. Horizon Therapeutics, Inc., IPR2016-00829.
`
`Jennifer Seminara, et al., “Establishing a consortium for the study
`of rare diseases: The Urea Cycle Disorders Consortium,” Molecular
`Genetics and Metabolism 100:S97-S105 (2010). (“Seminara”).
`
`RESERVED.
`
`Keith K. Vaux, “Book Reviews: A Clinical Guide to Inherited
`Metabolic Diseases, 2nd ed.,” J. Heredity 94:195 (2) (2003).
`(“Vaux Book Review”).
`
`RESERVED.
`
`RESERVED.
`
`Marshall Summar, “Current Strategies for the Management of
`Neonatal Urea Cycle Disorders,” J. Pediatrics 138:S30–S39 (2001).
`(“Summar 2001”).
`
`RAVICTI® product insert,
`http://www.accessdata.fda.gov/drugsatfda_docs/label/2016/203284s
`004lbl.pdf. (“Ravicti”).
`
`Marshall L. Summar, et al., “The Incidence of Urea Cycle
`Disorders,” Molecular Genetics and Metabolism 110:179–180
`(2013). (“Summar 2013”).
`
`Marshall L. Summar, et al., “Diagnosis, Symptoms, Frequency and
`Mortality of 260 Patients with Urea Cycle Disorders from a 21-
`Year, Multicentre Study of Acute Hyperammonaemic Episodes,”
`Acta Paediatrica 97:1420–25 (2008). (“Summar 2008”).
`
`IPR2017-01159
`
`
`
`Page 4
`
`
`
`Bridget Wilcken, “Problems in the Management of Urea Cycle
`Disorders,” Molecular Genetics and Metabolism 81:S86–S91
`(2004). (“Wilcken”).
`
`Information About FDA-Approved Drug, Buphenyl,
`http://www.accessdata.fda.gov/scripts/cder/daf/ (search Drug Name,
`Active Ingredient, or Application Number field for “020572”)
`
`2044
`
`2045
`
`
`
`
`Respectfully submitted,
`
` By: / M.C. Phillips /
`Matthew C. Phillips
`Registration No. 43,403
`Backup Counsel for Patent Owner
`
`
`Date: July 24, 2017
`
`
`
`
`
`
`
`
`
`
`
`
`IPR2017-01159
`
`
`
`Page 5
`
`
`
`CERTIFICATE OF SERVICE
`
`I hereby certify that on July 24, 2017, copies of the foregoing PATENT
`
`OWNER’S INITIAL EXHIBIT LIST and all documents filed with it were served
`
`via electronic mail, as agreed to by counsel, upon the following counsel for the
`
`Petitioners:
`
`Elizabeth J. Holland: eholland@goodwinlaw.com
`Cynthia Lambert Hardman: chardman@goodwinlaw.com
`
`
`Dated: July 24, 2017
`
`
`
`
`
`By: / M.C. Phillips /
`Matthew C. Phillips
`Registration No. 43,403
`Backup Counsel for Patent Owner
`
`
`
`IPR2017-01159
`
`
`
`Page 6
`
`