Prosecution Insights
Last updated: April 19, 2026
Application No. 18/267,591

ARIMOCLOMOL FOR THE TREATMENT OF NIEMANN PICK DISEASE, TYPE C, IN PATIENTS WITH ER TYPE MISSENSE MUTATIONS

Final Rejection §102§103§112
Filed
Jun 15, 2023
Examiner
CORNET, JEAN P
Art Unit
1628
Tech Center
1600 — Biotechnology & Organic Chemistry
Assignee
Zevra Denmark A/S
OA Round
2 (Final)
42%
Grant Probability
Moderate
3-4
OA Rounds
3y 1m
To Grant
90%
With Interview

Examiner Intelligence

Grants 42% of resolved cases
42%
Career Allow Rate
494 granted / 1171 resolved
-17.8% vs TC avg
Strong +48% interview lift
Without
With
+47.9%
Interview Lift
resolved cases with interview
Typical timeline
3y 1m
Avg Prosecution
69 currently pending
Career history
1240
Total Applications
across all art units

Statute-Specific Performance

§101
0.9%
-39.1% vs TC avg
§103
47.1%
+7.1% vs TC avg
§102
16.0%
-24.0% vs TC avg
§112
16.1%
-23.9% vs TC avg
Black line = Tech Center average estimate • Based on career data from 1171 resolved cases

Office Action

§102 §103 §112
DETAILED ACTION Notice of Pre-AIA or AIA Status The present application, filed on or after March 16, 2013, is being examined under the first inventor to file provisions of the AIA . Priority The present application is filed pursuant to 35 U.S.C. 371 as a U.S. National Phase application of International Patent Application No. PCT/EP2021/087483 which was filed December 23, 2021, claiming the benefit of U.S. Provisional Application No. 63/130,438, filed December 24, 2020, EP Application 21155579.2, filed February 5, 2021 and U.S. Provisional Application No. 63/185,111, filed May 6, 2021. Information Disclosure Statement The information disclosure statement filed 12/10/2025 and 01/07/2026 fails to comply with 37 CFR 1.98(a)(2), which requires a legible copy of each cited foreign patent document; each non-patent literature publication or that portion which caused it to be listed; and all other information or that portion which caused it to be listed. It has been placed in the application file, but the information referred to therein has not been considered. Simply citing "counterpart" foreign application an IDS without listing the actual references and their details (publication number, date, title, etc.) is not enough for proper disclosure; you need to list the specific citations, potentially with translations or concise explanations, to satisfy the duty of disclosure to the United States Patent and Trademark Office (USPTO) and avoid issues, especially if the foreign office cited them recently. Citing the counterpart application only provides a general reference, but the examiner needs the actual information (the prior art documents) to review for patentability. Copies of the references cited in the Japanese Patent Office have not been provided. Status of Claims Acknowledgement is made of the receipt and entry of the amendment to the claims filed on December 22, 2025. Claims 1-64 are canceled. Claims 65-95 are pending and under examination. Action Summary Claim 76 rejected under 35 U.S.C. 112(b) or 35 U.S.C. 112 (pre-AIA ), second paragraph, is withdraw as Applicant interpreted the “/” in Applicant’s remarks/argument filed on 12/22/2025 to be “and/or.” Claims 65-95 rejected under 35 U.S.C. 103 as being unpatentable over NCT02612129 (clinical trial.org, 08/25/2020) in view of Ingemann et al (Abstract/Molecular Genetics and Metabolism, Academic Press, Amsterdam, vol. 129, No. 2, January 31, 2020) are maintained. Claim Rejections - 35 USC § 103 In the event the determination of the status of the application as subject to AIA 35 U.S.C. 102 and 103 (or as subject to pre-AIA 35 U.S.C. 102 and 103) is incorrect, any correction of the statutory basis (i.e., changing from AIA to pre-AIA ) for the rejection will not be considered a new ground of rejection if the prior art relied upon, and the rationale supporting the rejection, would be the same under either status. The following is a quotation of 35 U.S.C. 103 which forms the basis for all obviousness rejections set forth in this Office action: A patent for a claimed invention may not be obtained, notwithstanding that the claimed invention is not identically disclosed as set forth in section 102, if the differences between the claimed invention and the prior art are such that the claimed invention as a whole would have been obvious before the effective filing date of the claimed invention to a person having ordinary skill in the art to which the claimed invention pertains. Patentability shall not be negated by the manner in which the invention was made. The factual inquiries for establishing a background for determining obviousness under 35 U.S.C. 103 are summarized as follows: 1. Determining the scope and contents of the prior art. 2. Ascertaining the differences between the prior art and the claims at issue. 3. Resolving the level of ordinary skill in the pertinent art. 4. Considering objective evidence present in the application indicating obviousness or nonobviousness. Claims 65-95 stand rejected under 35 U.S.C. 103 as being unpatentable over NCT02612129 (clinical trial.org, 08/25/2020) in view of Ingemann et al (Abstract/Molecular Genetics and Metabolism, Academic Press, Amsterdam, vol. 129, No. 2, January 31, 2020). Both references are cited in the IDS dated 09/13/2023. NCT02612129 teaches a method of treating With Niemann-Pick Disease Type C to a patient, the method comprising administering three times a day 100 mg capsule arimoclomol in combination with a therapeutically effective amount of miglustat to a patient subject, wherein the subject is identified as having one mutation in one allele of NPC1 and NPC2. (See Title, Aim Section, and Arm and Intervention Section.) The 100 mg 3x daily results 300 mg/day as recited in claims 77-79, 83-84, and 93-94. The 100 mg reads on claims 80 and 81. The daily administration reads on at least seven days a week of claim 82. A missense mutation in the NPC1 gene is an ER-type mutation. Accordingly, while the prior art does not teach miglustat contains N-alkyl derivative of 1,5-dideoxy-1,5-imino- D-glucitol in which said alkyl contains from 2-8 carbon atoms and arimoclomol is (+)-(R)-N-[2-hydroxy-3-(1-piperidinyl)- propoxy]-pyridine-1-oxide-3-carboximidoyl chloride citrate, miglustat contains N-alkyl derivative of 1,5-dideoxy-1,5-imino- D-glucitol in which said alkyl contains from 2-8 carbon atoms and arimoclomol is (+)-(R)-N-[2-hydroxy-3-(1-piperidinyl)- propoxy]-pyridine-1-oxide-3-carboximidoyl chloride citrate absent evidence to the contrary. A chemical compound and its properties are inseparable. In re Spada, 911 F.2d 705, 709, 15 USPQ2d 1655, 1658 (Fed. Cir. 1990). NCT02612129 does not teach the mutation in the NPC1 gene is ER (endoplasmic reticulum) type missense mutation, wherein the ER type missense mutation is I1061T. Additionally, NCT02612129 does not teach the patient is a human (mammal). Ingemann teaches Niemann-Pick disease type C (NPC) is a rare and devastating neurodegenerative disease, caused by mutations in either the NPC1 (95% of cases) or NPQ genes. NPC1 is a lysosomal/endosomal membrane protein playing an essential role in lysosomal biogenesis and lipid transport. NPC disease is characterized by a dysfunctional lysosomal compartment and aberrant accumulation of cholesterol and other lipids inside the cells of multiple tissues. Three patients had severe mutations on both alleles of NPC1, leading to little or no functional NPC1 protein. In NPC fibroblast cell lines covering the most prominent genetic NPC1 mutations found in the clinical trial, arimoclomol treatment increased the amount of properly processed NPC1 protein. This included a well described ER retention mutant (!1061 T) as well as a mutation known to display lysosomal localization (P1007A), induced increased expression of HSP70 and reduced accumulation of unesterified cholesterol in peripheral blood mononuclear cells, as well as reduced the cholestane-triol levels in serum. (See Abstract.) It would have been prima facie obvious to one of ordinary skill in the art at the time the invention was filed to modify the method taught by NCT02612129 by including a human subject that is identified as having one ER type missense mutation in the NPC allele, in this case I1061T mutation to give Applicant’s claimed invention. One would have been motivated to do so, because NCT02612129 discloses a patient that embraces a human (mammal) patient and also because Ingemann taches arimoclomol treatment can increase the amount of properly processed NPC1 protein where said protein can be an ER mutant (I1061T). One would reasonably expect the method of NCT02612129 to effective treat Niemann-Pick disease type C (NPC) in a human subject that is identified as having one ER type missense mutation in the NPC allele, in this case I1061T mutation, successfully. Acknowledgement is made of the receipt and entry of Applicant’s remarks/arguments filed on December 09, 2024. Applicant’s argument Applicant argue that the Ingemann et al. abstract is not eligible to serve as the basis of a novelty or obviousness rejection under 35 U.S.C. §102(b)(1)(B) because credited authors Christine Dali, Torp Peterson, and Nikolaj Havnsoe are joint inventors of the instant application and the disclosure was made within one year of the instant application's claimed priority date. The instant application claims priority to U.S. Provisional Application 63/130,438 which was filed on December 24, 2020. The one-year grace-period for disclosures made by a j oint inventor extends back to December 24, 2019. The Ingemann et al. abstract was published on February 10, 2020, within said 1-year grace period, and therefore cannot form the basis of novelty or obviousness rejections Examiner’s response In response, Applicant’s argument is not persuasive. Specifically, the Ingemann’s references cited 6 authors namely, Linda Ingemann, Nikolaj Havnsoe Torp Petersen (present inventor), Anja Koutrup, Catherine Kolster Fog, Arnela Mehmedbasic, and Christine Dalli (present invention). Linda Ingemann, Anja Koutrup, Catherine Kolster Fog, and Arnela Mehmedbasic are not joint inventors. Subsequently, 35 U.S.C. §102(b)(1)(B) exception states “the subject matter disclosed had, before such disclosure, been publicly disclosed by the inventor, or a joint inventor, or another who obtained the subject matter disclosed directly or indirectly from the inventor or a joint inventor.” The term “joint inventor” refers to any one of the individuals who invented or discovered the subject matter of a joint invention. In the present case, the fact that there are four authors that did not invent or discover the subject matter of a joint invention, these authors are not joint inventors. Therefore, 35 U.S.C. §102(b)(1)(B) exception does not apply. Ingemann is prior art under 35 U.S.C. 102(a)(1). Furthermore, Ingemann is cited in the Japanese Patent Office cited in the IDS 01/07/2026 as “Ingemann, Linda, Rescue of NPC1 protein and effect on biomarkers by arimoclomol treatment in Niemann-Pick disease type C, Molecular Genetics and Metabolism, 2019, Vol, 129, No. 2, S78” with a publication year 2019. This document appears to be the same document used in the office action that was previously cited in the IDS dated 09/14/2023 with a publication year of January 31, 2020. To the extent this is the same document, the exception does not apply as well as the year 2019 is considered by the Examiner to be more than one year unless Applicant can prove otherwise. Applicant’s argument Applicant argue that the NCT02612129 study publication fails to disclose ER-type missense mutations in the NPC1 and/or NPC2 genes. Therefore, a person skilled in the art would have no reasonable expectation of successfully treating patients with Niemann-Pick disease type C attributed to ER- type missense mutations using N-[2-hydroxy-3-(1-piperidinyl)-propoxy]-pyridine-1-oxide-3- carboximidoyl chloride. Examiner’s response In response, Applicant’s argument is not persuasive because NCT02612129 clearly discloses ER-type missense mutations in the NPC1 and/or NPC2 genes. However, NCT02612129 does not teach the NPC1 genotype is 11061T and said genotype is taught by Ingemann. a person skilled in the art would have a reasonable expectation of successfully treating patients with Niemann-Pick disease type C attributed to ER- type missense mutations using N-[2-hydroxy-3-(1-piperidinyl)-propoxy]-pyridine-1-oxide-3- carboximidoyl chloride. Conclusion Claims 65-95 are not allowed. THIS ACTION IS MADE FINAL. Applicant is reminded of the extension of time policy as set forth in 37 CFR 1.136(a). A shortened statutory period for reply to this final action is set to expire THREE MONTHS from the mailing date of this action. In the event a first reply is filed within TWO MONTHS of the mailing date of this final action and the advisory action is not mailed until after the end of the THREE-MONTH shortened statutory period, then the shortened statutory period will expire on the date the advisory action is mailed, and any nonprovisional extension fee (37 CFR 1.17(a)) pursuant to 37 CFR 1.136(a) will be calculated from the mailing date of the advisory action. In no event, however, will the statutory period for reply expire later than SIX MONTHS from the mailing date of this final action. Any inquiry concerning this communication or earlier communications from the examiner should be directed to JEAN P CORNET whose telephone number is (571)270-7669. The examiner can normally be reached Monday-Thursday from 7.00am-5.30pm. Examiner interviews are available via telephone, in-person, and video conferencing using a USPTO supplied web-based collaboration tool. To schedule an interview, applicant is encouraged to use the USPTO Automated Interview Request (AIR) at http://www.uspto.gov/interviewpractice. If attempts to reach the examiner by telephone are unsuccessful, the examiner’s supervisor, Amy L Clark can be reached at 571-272-1310. The fax phone number for the organization where this application or proceeding is assigned is 571-273-8300. Information regarding the status of published or unpublished applications may be obtained from Patent Center. Unpublished application information in Patent Center is available to registered users. To file and manage patent submissions in Patent Center, visit: https://patentcenter.uspto.gov. Visit https://www.uspto.gov/patents/apply/patent-center for more information about Patent Center and https://www.uspto.gov/patents/docx for information about filing in DOCX format. For additional questions, contact the Electronic Business Center (EBC) at 866-217-9197 (toll-free). If you would like assistance from a USPTO Customer Service Representative, call 800-786-9199 (IN USA OR CANADA) or 571-272-1000. /JEAN P CORNET/Primary Examiner, Art Unit 1628
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Prosecution Timeline

Jun 15, 2023
Application Filed
Sep 18, 2025
Non-Final Rejection — §102, §103, §112
Dec 22, 2025
Response Filed
Jan 09, 2026
Final Rejection — §102, §103, §112 (current)

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Prosecution Projections

3-4
Expected OA Rounds
42%
Grant Probability
90%
With Interview (+47.9%)
3y 1m
Median Time to Grant
Moderate
PTA Risk
Based on 1171 resolved cases by this examiner. Grant probability derived from career allow rate.

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