ACKR3

DONGTAN, KOREA, June 10, 2024 - iLeadBMS, a new drug R&D company under Ildong Pharmaceutical Group, announced on the 10th that it presented in vivo study results on its liver fibrosis treatment candidate 'IL1512' at the European Association for the Study of the Liver (EASL) 2024, held in Milan, Italy, from June 5th to the 8th. IL1512 is an oral CXCR7 agonist and is classified as a first-in-class drug, a type of drug that has never been developed for the same therapeutic indication previously. Last month, iLeadBMS presented the efficacy results of this drug for idiopathic pulmonary fibrosis (IPF) at the American Thoracic Society (ATS 2024), and this time around it announced the anti-fibrotic effects of the drug in the liver. CXCR7 is a G protein-coupled receptor that binds to chemokine ligands CXCL11 and CXCL12, targeting various pathways related to fibrosis such as fibroblast activation, inflammation, tissue repair, and angiogenesis. This makes it an ideal therapeutic target for treating fibrotic diseases. Given that CXCR7 agonists exerts their anti-fibrotic properties through a novel mechanism, iLeadBMS plans to develop anti-fibrotic drugs to treat fibrosis in various organs including but not limited to the lungs, liver, heart, kidneys, and skin, and is also considering utilizing the expedited review and Orphan Drug Designation (ODD) pathways offered by the FDA. Based on the presentation at the conference, iLeadBMS has confirmed the anti-fibrotic effects of IL1512 in the well-established CCl4 (carbon tetrachloride) liver fibrosis model with oral administration of the test article. Significant improvements in various anti-fibrotic indicators (col 1a1, hydroxyproline content, TGF-beta) were observed compared to the placebo. The presentation emphasized that IL1512, with its novel mechanism targeting CXCR7, demonstrated direct anti-inflammatory effects on the liver and improvement in fibrosis. Additionally, it highlighted that IL1512 showed either superior or at minimum, comparable efficacy and safety profiles compared to Elafibranor, the comparator drug. Based on these research results, iLeadBMS has identified preclinical candidate molecules with even greater improvements over IL1512 and, plans to start GLP (Good Laboratory Practice) toxicology studies in the second half of this year. About iLeadBMS Established in 2020, iLeadBMS specializes in the discovery and development of novel drugs, with an R&D pipeline targeting fibrotic diseases, protease inhibitors, solid tumors, and neurodegenerative diseases. For more information on iLeadBMS, please visit:

DONGTAN, KOREA, May 21, 2024 – iLeadBMS, a leading biotech company dedicated to the discovery and development of novel drugs for high unmet medical needs, has announced a positive outcome of IL1512, a first-in-class CXCR7 agonist, as a potential therapy for pulmonary fibrotic diseases at the American Thoracic Society (ATS 2024) conference held in San Diego from May 17th to 22nd. Idiopathic pulmonary fibrosis (IPF) is a rare lung disease characterized by progressive fibrosis of lung parenchyma, with a less than 40% survival rate five years after diagnosis. Research And Markets projects the IPF treatment market to reach approximately $6.1 billion (about 8 trillion KRW) by 2030, indicating significant unmet medical needs in this area. To address these high unmet needs, iLeadBMS has concentrated its research on CXCR7 (C-X-C chemokine receptor 7), a key mediator in signal transduction amplifying fibrosis and inflammation. CXCR7 selectively binds to chemokine ligands CXCL11 and CXCL12, targeting various pathways associated with fibrosis, inflammation, tissue repair, and angiogenesis. IL1512 has exhibited high selectivity for CXCR7 without binding to other chemokine receptors and demonstrated promising pharmacokinetics (DMPK) in mice and rats when orally administered. In a bleomycin-induced pulmonary fibrosis model, IL1512 showed dose-dependent improvements in the Ashcroft score, confirming its potentially superior anti-fibrotic efficacy compared to the standard therapy. Additionally, IL1512 displayed a favorable safety pro by the absence of weight loss associated with drug administration. Based on these results, it is anticipated that IL1512 will have a better safety and efficacy pro compared to currently available standard treatments. Preclinical studies for the selected CXCR7 agonist candidate have been initiated and GLP toxicity studies are planned for the second half of this year. The Company’s strategy aims to develop therapies with minimal side effects while providing broad anti-fibrotic effects, covering multiple organs beyond just the lungs. It is anticipated that expedited regulatory pathways such as Orphan Drug Designation (ODD) will be considered for the fibrosis program and concurrently, CXCR7 agonists with blood-brain barrier penetration potential, are also being explored for neurological disorders. About iLeadBMS Established in 2020, iLeadBMS specializes in the discovery and development of novel drugs, with an R&D pipeline targeting fibrotic diseases, protease inhibitors, solid tumors, and neurodegenerative diseases. For more information on iLeadBMS, please visit:

Ad hoc announcement pursuant to Art. 53 LR CEO, Jean-Paul Clozel, to update on how Idorsia is being adapted for sustainable value creationThe company is focused on extending the cash runway beyond the current estimate of early April 2024, through various avenues, including potential out-licensing dealsPostponement of the publication of Full-Year 2023 Financial Results and the subsequent Annual General Meeting of Shareholders Allschwil, Switzerland – January 10, 2024Idorsia Ltd (SIX: IDIA) today announced that Jean-Paul Clozel, Chief Executive Officer of Idorsia, will present at the J.P. Morgan Healthcare Conference on January 10, 2024, at 10:30 PST / 19:30 CET. The conference will take place at the Westin St. Francis hotel in San Francisco, USA. Jean-Paul will describe how Idorsia is being adapted for sustainable value creation. The presentation will cover the progress of QUVIVIQ™ (daridorexant) in the US and Europe, and the clinical data included in the new drug application for aprocitentan with the US Food and Drug Administration (FDA) which is currently under review. He will also present other unencumbered assets that provide the company with strategic flexibility and several avenues to explore potential fundraising. Follow this link to access the audio stream and find the presentation available here. Jean-Paul Clozel MD and Chief Executive Officer of Idorsia commented: “Creating a sustainable pharma company requires scientific innovation and substantial investment. We have demonstrated our ability to innovate and bring new drugs to the market and have a portfolio of innovative products, however, we have limited financing, so we need to prioritize activities that offer the maximum return in the near term. We estimate the current cash reserves to last to early April 2024, we therefore plan to extend the cash runway through various avenues, including potential out-license deals.” Expected highlights in 2024 Secure additional funding to extend the cash runwayExpand access and availability of QUVIVIQ (daridorexant) in the US, Canada and across EuropeAchieve FDA decision for the NDA for aprocitentan in resistant hypertensionAchieve EMA decision for the MAA for aprocitentan in resistant hypertension Jean-Paul commented on the portfolio: “More than 11 million QUVIVIQ tablets have been dispensed to help patients achieve better nights and days. We have made great progress with access and availability in our key markets, and I expect to see that translating into income in 2024. I also expect aprocitentan – the first antihypertensive working on a new pathway for 30 years – to become available for treated patients whose hypertension remains uncontrolled. Discussions with health authorities are going well and I’m confident that the label will reflect the outstanding results we’ve seen for these high-risk patients. We also intend to progress the two global Phase 3 programs with selatogrel and cenerimod which have the potential to transform treatment in their target indications.” Idorsia’s portfolio Product / compoundMechanism of actionTherapeutic areaStatusQUVIVIQ™ (daridorexant)Dual orexin receptor antagonistInsomniaCommercially available in the US Germany, Italy, Switzerland, Spain, the UK, and Canada; Approved in the EU; Phase 2 in pediatric insomnia – recruitingPartners: Mochida & Sosei in Japan, Simcere in China and Hong KongAprocitentanDual endothelin receptor antagonistResistant hypertensionNDA under review in the US, MAA under review in the EU, other filings in preparationPartner: UnencumberedLucerastatGlucosylceramide synthase inhibitorFabry diseasePhase 3 primary endpoint not met; open-label extension study ongoingPartner: UnencumberedSelatogrelP2Y12 inhibitorSuspected acute myocardial infarctionPhase 3 recruitingPartner: UnencumberedCenerimodS1P1 receptor modulatorSystemic lupus erythematosusPhase 3 recruitingPartner: UnencumberedACT-1004-1239ACKR3 / CXCR7 antagonistMultiple sclerosis and other demyelinating diseasesPhase 2 in preparationPartner: UnencumberedSinbaglustatGBA2/GCS inhibitorRare lysosomal storage disordersPhase 1 completePartner: UnencumberedACT-1014-6470C5aR1 antagonistImmune-mediated disordersPhase 1Partner: UnencumberedACT-777991CXCR3 antagonistRecent-onset Type 1 diabetesPhase 1Partner: UnencumberedIDOR-1117-2520UndisclosedImmune-mediated disordersPhase 1Partner: UnencumberedIDO-090Synthetic glycan vaccineClostridium difficile infectionPhase 1 in preparationPartner: Unencumbered Neurocrine Biosciences has a global license to develop and commercialize ACT-709478 (NBI-827104), Idorsia's novel T-type calcium channel blocker. On July 20, 2023, Idorsia sold its operating businesses in the Asia Pacific (ex-China) region to Sosei Heptares, including the assignment of the license for PIVLAZ (clazosentan) for the Asia Pacific (ex-China) region. Idorsia retains the rights to clazosentan in the rest of the world. Further details including the current status of each project in our portfolio can be found in our innovation fact sheet. Upcoming Financial UpdatesThe company has decided to postpone the publication of Full-Year 2023 Financial Results and the subsequent Annual General Meeting of Shareholders. Full-Year 2023 and First Quarter 2024 Financial Results reporting on April 25, 2024 Annual General Meeting of Shareholders on June 13, 2024 Half-Year 2024 Financial Results reporting on July 25, 2024 Notes to the editor About IdorsiaIdorsia Ltd is reaching out for more – We have more ideas, we see more opportunities and we want to help more patients. In order to achieve this, we will develop Idorsia into a leading biopharmaceutical company, with a strong scientific core. Headquartered near Basel, Switzerland – a European biotech-hub – Idorsia is specialized in the discovery, development and commercialization of small molecules to transform the horizon of therapeutic options. Idorsia has a 20-year heritage of drug discovery, a broad portfolio of innovative drugs in the pipeline, an experienced team of professionals covering all disciplines from bench to bedside, and commercial operations in Europe and North America – the ideal constellation for bringing innovative medicines to patients. Idorsia was listed on the SIX Swiss Exchange (ticker symbol: IDIA) in June 2017 and has over 800 highly qualified specialists dedicated to realizing our ambitious targets. For further information, please contactAndrew C. WeissSenior Vice President, Head of Investor Relations & Corporate CommunicationsIdorsia Pharmaceuticals Ltd, Hegenheimermattweg 91, CH-4123 Allschwil+41 58 844 10 10investor.relations@idorsia.commedia.relations@idorsia.com www.idorsia.com The above information contains certain "forward-looking statements", relating to the company's business, which can be identified by the use of forward-looking terminology such as "estimates", "believes", "expects", "may", "are expected to", "will", "will continue", "should", "would be", "seeks", "pending" or "anticipates" or similar expressions, or by discussions of strategy, plans or intentions. Such statements include descriptions of the company's investment and research and development programs and anticipated expenditures in connection therewith, descriptions of new products expected to be introduced by the company and anticipated customer demand for such products and products in the company's existing portfolio. Such statements reflect the current views of the company with respect to future events and are subject to certain risks, uncertainties and assumptions. Many factors could cause the actual results, performance or achievements of the company to be materially different from any future results, performances or achievements that may be expressed or implied by such forward-looking statements. Should one or more of these risks or uncertainties materialize, or should underlying assumptions prove incorrect, actual results may vary materially from those described herein as anticipated, believed, estimated or expected. Attachment Press Release PDF