Clazosentan sodium

Vamorolone is approved and marketed as AGAMREE® for the treatment of Duchenne Muscular Dystrophy (DMD) in the US, European Union, UK and China New five-year data on vamorolone highlighted substantially improved safety pro reduced vertebral fractures, lower incidence of cataracts and that normal growth rate was maintained. With adverse effects being a key limitation of conventional corticosteroid therapy, vamorolone’s safety pro its comparable effectiveness, has the potential to displace existing steroid treatments Transaction advances Nxera’s mission to bring innovative medicines to patients in Japan/APAC in line with 2030 vision to build high-growth, highly profitable Japanese biopharma company Tokyo, Japan and Cambridge, UK, 8 January 2026 – Nxera Pharma Co. Ltd (“Nxera” or “the Company; TSE 4565) today announces that it has entered an exclusive licensing agreement for the development, manufacturing and commercialization of vamorolone for the treatment of Duchenne Muscular Dystrophy (DMD) in Japan, South Korea, Australia and New Zealand with Santhera Pharmaceuticals Holding (“Santhera”) (SIX: SANN). Vamorolone is approved and marketed as AGAMREE® for the treatment of DMD, a rare inherited neurodegenerative disease, in the US, European Union, UK and China. The addition of vamorolone brings into Nxera’s portfolio of innovative medicines for rare and specialty diseases, a late-stage development candidate with the potential to address significant unmet needs of patients in Japan and the Asia-Pacific (“APAC”) region living with DMD. This portfolio includes PIVLAZ™ (clazosentan) and QUVIVIQ™ (daridorexant), both of which are approved and marketed in Japan and under commercial development in other APAC countries. Under the terms of the agreement, Nxera will be responsible for obtaining regulatory approval of vamorolone in the licensed territories, including undertaking regulatory activities such as clinical trials where required, and will lead commercialization and manufacturing activities for those territories. This strategic partnership leverages Nxera's proven commercial capabilities and development and regulatory expertise across the APAC region, particularly in Japan. In addition, Nxera’s team brings significant prior development and manufacturing experience with vamorolone through its 2023 acquisition of Idorsia’s Japan and APAC business. Christopher Cargill, President and Chief Executive Officer of Nxera, commented: “We are excited to partner with Santhera to bring vamorolone to DMD patients in Japan, South Korea, Australia and New Zealand. Vamorolone’s differentiated safety and efficacy pro the potential to fundamentally change the standard of care by enabling early use, full dosing, and long-term treatment, addressing critical limitations of existing steroid therapies currently used in the region. “Furthermore, the transaction expands our portfolio of late- and commercial-stage products for Japan/APAC and advances our mission to bring innovative medicines to patients in these important regions in line with our 2030 vision to build high-growth, highly profitable Japanese biopharma company.” Dario Eklund, Chief Executive Officer of Santhera, added: “This strategic partnership represents a significant milestone in our mission to expand global access to AGAMREE® (vamorolone) and bring meaningful new treatment options to patients with DMD worldwide. Nxera's deep expertise and established infrastructure in Japan and across the wider APAC region, as well as prior experience of vamorolone, make them an ideal partner to unlock the full commercial and clinical potential of AGAMREE® (vamorolone) in these markets and accelerate access for patients with DMD.” Additional transaction terms In return for development, manufacturing and commercialization rights for vamorolone in the licensed territories, Nxera will make an upfront payment of USD 40 million to Santhera, consisting of USD 30 million in cash and USD 10 million as a strategic equity investment. Santhera is also eligible to receive sales and regulatory milestone payments of up to USD 165 million from Nxera as well as tiered royalties starting in the low teens on net sales of vamorolone in the licensed territories. About Duchenne Muscular Dystrophy Duchenne muscular dystrophy (DMD) is a rare inherited X-chromosome-linked disease, which almost exclusively affects males. DMD is characterized by inflammation which is present at birth or shortly thereafter. Inflammation leads to fibrosis of muscle and is clinically manifested by progressive muscle degeneration and weakness. Major milestones in the disease are the loss of ambulation, the loss of self-feeding, the start of assisted ventilation, and the development of cardiomyopathy. DMD reduces life expectancy to before the fourth decade due to respiratory and/or cardiac failure. Corticosteroids are the current standard of care for the treatment of DMD. About vamorolone Vamorolone is a novel drug with a mode of action based on binding to the same receptor as glucocorticoids but modifying its downstream activity. Moreover, it is not a substrate for the 11-β-hydroxysteroid dehydrogenase (11β-HSD) enzymes that may be responsible for local drug amplification and corticosteroid-associated toxicity in local tissues [1-4]. This mechanism has shown the potential to ‘dissociate’ efficacy from steroid safety concerns and therefore vamorolone is positioned as a dissociative anti-inflammatory drug and an alternative to existing corticosteroids, the current standard of care in children and adolescent patients with DMD [1-4]. In the pivotal VISION-DMD study, vamorolone met the primary endpoint Time to Stand (TTSTAND) velocity versus placebo (p=0.002) at 24 weeks of treatment and showed a good safety and tolerability profile [1, 4]. The most commonly reported side effects were cushingoid features, vomiting, weight increase and irritability. Side effects were generally of mild to moderate severity. Five-year data from the GUARDIAN study highlighted substantially improved safety pro reduced vertebral fractures, lower incidence of cataracts and that normal growth rate was maintained. Currently available data show that vamorolone, unlike corticosteroids, has no restriction of growth [5] and no negative effects on bone metabolism as demonstrated by normal bone formation and bone resorption serum markers [6]. ▼ This medicinal product is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions. References [1] Dang UJ et al. (2024) Neurology 2024;102:e208112. doi.org/10.1212/WNL.0000000000208112. Link . [2] Guglieri M et al (2022). JAMA Neurol. 2022;79(10):1005-1014. doi:10.1001/jamaneurol.2022.2480 Link . [3] Liu X et al (2020). Proc Natl Acad Sci USA 117:24285-24293 [4] Heier CR et al (2019). Life Science Alliance DOI: 10.26508 [5] Ward et al., WMS 2022, FP.27 - Poster 71. Link . [6] Hasham et al., MDA 2022 Poster presentation. Link . –END– About Nxera Pharma Nxera Pharma is a technology powered biopharma company in pursuit of new specialty medicines to improve the lives of patients with unmet needs in Japan and globally. The Company has built an agile, new-generation commercial business in Japan to develop and commercialize innovative medicines, including several launched products, to address this high-value, large and growing market and those in the broader APAC region. In addition, and powered by its unique NxWave™ GPCR structure-based drug discovery platform, the Company is advancing an extensive pipeline internally and in partnership with leading pharma and biotech companies. Nxera Pharma operates at key locations in Tokyo and Osaka (Japan), London and Cambridge (UK), Basel (Switzerland) and Seoul (South Korea) and is listed on the Tokyo Stock Exchange (ticker: 4565). For more information, please visit LinkedIn: @NxeraPharma | X: @NxeraPharma | YouTube: @NxeraPharma QUVIVIQ™ is trademark of Idorsia Ltd. About Santhera Santhera Pharmaceuticals (SIX: SANN) is a Swiss specialty pharmaceutical company focused on the development and commercialization of innovative medicines for rare neuromuscular diseases with high unmet medical need. The Company has an exclusive license from ReveraGen for all indications worldwide to AGAMREE® (vamorolone), a dissociative steroid with novel mode of action, which was investigated in a pivotal study in patients with Duchenne muscular dystrophy (DMD) as an alternative to standard corticosteroids. AGAMREE for the treatment of DMD is approved in the U.S. by the Food and Drug Administration (FDA), in the EU by the European Commission (EC), in the UK by the Medicines and Healthcare products Regulatory Agency (MHRA), in China by the National Medical Products Administration (NMPA), in Hong Kong by the Department of Health (DoH) and in Canada by Health Canada. Santhera has out-licensed rights to AGAMREE for North America to Catalyst Pharmaceuticals and for China and certain countries in Southeast Asia to Sperogenix Therapeutics. For further information, please visit . AGAMREE® is a trademark of Santhera Pharmaceuticals. Enquiries: For Nxera Pharma Media and Investor Relations Shinya Tsuzuki, VP, Head of Investor Relations Maya Bennison, Communications Manager +81 (0)3 5962 5718 | +44 (0)1223 949390 | IR@Nxera.life MEDiSTRAVA (for International Media) Mark Swallow, Frazer Hall, Erica Hollingsworth +44 (0)203 928 6900 | Nxera@medistrava.com For Santhera Catherine Isted, Chief Financial Officer IR@santhera.com ICR Healthcare Santhera@icrhealthcare.com Forward-looking statements This press release contains forward-looking statements, including statements about the discovery, development, and commercialization of products. Various risks may cause Nxera Pharma Group’s actual results to differ materially from those expressed or implied by the forward looking statements, including: adverse results in clinical development programs; failure to obtain patent protection for inventions; commercial limitations imposed by patents owned or controlled by third parties; dependence upon strategic alliance partners to develop and commercialize products and services; difficulties or delays in obtaining regulatory approvals to market products and services resulting from development efforts; the requirement for substantial funding to conduct research and development and to expand commercialization activities; and product initiatives by competitors. As a result of these factors, prospective investors are cautioned not to rely on any forward-looking statements. We disclaim any intention or obligation to update or revise any forward-looking statements, whether as a result of new information, future events or otherwise.

Tokyo, Japan and Cambridge, UK, 8 August 2025 – Nxera Pharma (“the Company” or “Nxera”; TSE: 4565) provides an update on operational activities and reports its consolidated results for the second quarter and first half ended 30 June 2025. The full report can be found here. Chris Cargill, President and CEO of Nxera, commented: “Nxera continued to make good progress in the first half of 2025. We are seeing increasing revenues from sales of PIVLAZ® and QUVIVIQ®, our marketed products in Japan, as well as notable progress across our partnered programs, with multiple programs advancing through clinical development and reaching important revenue-bearing milestones. This includes NBI-‘568 – being advanced by Neurocrine – which is the first candidate designed using Nxera technology to enter Phase 3 trials. Using our NxWave™ platform, these candidates are designed to address areas of significant and increasing medical need across our key therapeutic focus areas of obesity and metabolic disorders, neurology/neuropsychiatry, and immunology and inflammation. In addition, we continue to make progress advancing our inhouse pipeline of novel candidates. Moving into the second half of 2025, we anticipate further key data readouts from multiple clinical trials and with several new studies due to commence, we are well positioned to deliver significant value for patients and shareholders.” Operational Highlights for Q2 2025 Progress with marketed products in Japan The Group sells PIVLAZ® (clazosentan sodium 150mg) for the prevention of cerebral vasospasm in Japan using its in-house salesforce. PIVLAZ® revenue increased from JPY 5,393 million to JPY 5,805 million (up by 7.6% vs the prior corresponding period)The Group earns royalty revenue on sales of QUVIVIQ® (daridorexant 25 and 50 mg) by Shionogi & Co., Ltd. (“Shionogi”), as well as revenue on the supply of QUVIVIQ® to Shionogi. This amounted to JPY 1,586 million for the first half 2025. As sales of QUVIVIQ® began in the fourth quarter of the prior year, there were no sales for the prior corresponding period Progress with partnered programs Neurocrine Biosciences dosed the first patient in its Phase 3 registrational program of NBI-1117568 (NBI-‘568), resulting in a payment of US$15 million to the Company NBI-‘568 is an oral, selective muscarinic M4 receptor agonistThe Phase 3 study is a global double-blind, placebo-controlled trial evaluating NBI-’568 in adults with a primary diagnosis of schizophrenia who are experiencing an acute exacerbation or relapse of symptomsThe study is expected to enroll approximately 280 patients. The primary endpoint of the study is a reduction from baseline in the Positive and Negative Syndrome Scale (PANSS) The key secondary endpoint is improvement in the Clinical Global Impression of Severity (CGI-S) scale Development milestone achieved under the multi-target collaboration and license agreement with Eli Lilly and Company targeting diabetes and metabolic diseases, resulting in a payment to the Company US$4.8 million milestone payment received from Centessa Pharmaceuticals (UK) Limited (“Centessa”) as a result of Centessa initiating clinical development of ORX142 ORX142 is Centessa’s second investigational, novel, highly potent and selective OX2R agonist discovered using Nxera technology and being developed for the treatment of select neurological and neurodegenerative disordersThe milestones were achieved as Centessa first received clearance of an Investigational New Drug (IND) application by the US Food and Drug Administration (FDA) for a Phase 1 clinical study of ORX142 in healthy volunteers and second, with the initiation of the studyThe payment receipt was fully recognized as revenue in the second quarter of 2025 Post-period events Nxera launched a broad proprietary pipeline targeting obesity and chronic weight management Independent of our productive drug discovery collaborations with Pfizer and Eli Lilly, Nxera has established, expanded and accelerated drug discovery efforts of its own proprietary pipeline across a broad range of validated GPCR targets in obesity and associated metabolic disordersCentral to this pipeline is Nxera’s new, wholly-owned oral small molecule GLP-1 agonist program, focused on differentiated chemistry, which is distinct, independent and developed separately from Pfizer’s PF-06954522, allowing Nxera full control to drive rapid progressComplementing this program, Nxera is simultaneously accelerating the advancement of an additional six established GPCR-targeted programs focused on obesity and chronic weight managementPfizer discontinued development of its Phase 1 candidate PF-06954522 (a small molecule GLP-1 agonist, which was discovered under a strategic drug discovery collaboration with Nxera) due to a portfolio decision by Pfizer Financial Highlights for the Six-month Period ended 30 June 2025 Revenue totalled JPY 15,094 million (US$101.6 million*), an increase of JPY 2,374 million (US$18.0 million) vs. the prior corresponding period. This increase was primarily due to the inclusion of sales of QUVIVIQ® following its launch in the fourth quarter of 2024. R&D expenses totalled JPY 7,474 million (US$50.3 million), an increase of JPY 1,987 million (US$14.2 million) vs. the prior corresponding period. This increase primarily reflects an increased investment in R&D and the impact of the weaker Yen.SG&A expenses totalled JPY 7,566 million (US$51.0 million), a decrease of JPY 456 million (US$1.8 million) vs. the prior corresponding period. This decrease was primarily due to lower selling related costs as a result of targeted cost savings, partially offset by incremental spend on personnel to strengthen organizational capabilities.Operating loss totalled JPY 2,756 million (US$18.5 million) vs. an operating loss of JPY 3,654 million (US$24.0 million) in the prior corresponding period. This change in profitability reflects the combined effect of all movements explained above.Loss before income tax totalled JPY 3,722 million (US$25.1 million) vs. a loss before income tax of JPY 3,158 million (US$20.8 million) in the prior corresponding period.Net loss totalled JPY 3,137 million (US$21.1 million) vs. a net loss of JPY 4,703 million (US$30.9 million) in the prior corresponding period.Core operating profit** totalled JPY 364 million (US$2.5 million) vs. a core operating profit of JPY 1,176 million (US$7.7 million) in the prior corresponding period. Cash and cash equivalents as at 30 June 2025 amounted to JPY 32,997 million (US$228.1 million) having increased by JPY 729 million (US$22.3 million) from the beginning of the year. *Convenience conversion to US$ at the following rates: FY 2025: 1US$ =148.56 JPY; FY 2024: 1US$ =152.12 JPY; 30 Jun 2025: 1US$ = 144.66 JPY; 31 Dec 2024: 1US$ = 156.83 JPY ** Core operating profit / loss is an alternative performance measure which adjusts for material non-cash costs and one-off costs in order to provide insights into the recurring cash generation capability of the core business. –END– About Nxera PharmaNxera Pharma is a technology powered biopharma company, in pursuit of new specialty medicines to improve the lives of patients with unmet needs in Japan and globally. We have built an agile, new-generation commercial business in Japan to develop and commercialize innovative medicines, including several launched products, to address this high value, large and growing market and those in the broader APAC region. Behind that, and powered by our unique NxWave™ discovery platform, we are advancing an extensive pipeline of over 30 active programs from discovery through to late clinical stage internally and in partnership with leading pharma and biotech companies. This pipeline of potentially first- and best-in-class candidates is focused on addressing major unmet needs in some of the fastest-growing areas of medicine across obesity and metabolic disorders, neurology/neuropsychiatry and immunology and inflammation. Nxera employs approximately 400 talented people at key locations in Tokyo and Osaka (Japan), London and Cambridge (UK), Basel (Switzerland) and Seoul (South Korea) and is listed on the Tokyo Stock Exchange (ticker: 4565). For more information, please visit www.nxera.life LinkedIn: @NxeraPharma | X: @NxeraPharma | YouTube: @NxeraPharma Enquiries: Nxera – Media and Investor RelationsShinya Tsuzuki, VP, Head of Investor RelationsShinichiro Nishishita, VP Investor Relations, Head of Regulatory DisclosuresMaya Bennison, Communications Manager+81 (0)3 5210 3399 | +44 (0)1223 949390 |IR@Nxera.life MEDiSTRAVA (for International Media)Mark Swallow, Frazer Hall, Erica Hollingsworth+44 (0)203 928 6900 | Nxera@medistrava.com Forward-looking statementsThis press release contains forward-looking statements, including statements about the discovery, development, and commercialization of products. Various risks may cause Nxera Pharma Group’s actual results to differ materially from those expressed or implied by the forward looking statements, including: adverse results in clinical development programs; failure to obtain patent protection for inventions; commercial limitations imposed by patents owned or controlled by third parties; dependence upon strategic alliance partners to develop and commercialize products and services; difficulties or delays in obtaining regulatory approvals to market products and services resulting from development efforts; the requirement for substantial funding to conduct research and development and to expand commercialization activities; and product initiatives by competitors. As a result of these factors, prospective investors are cautioned not to rely on any forward-looking statements. We disclaim any intention or obligation to update or revise any forward-looking statements, whether as a result of new information, future events or otherwise. Attachment Nxera Pharma FY25Q2 E_Tanshin