Diffuse midline glioma, point mutation K27M in histone H3

- Addition of dordaviprone strengthens Jazz's late-stage oncology pipeline and reinforces commitment to addressing rare diseases with significant unmet need - DUBLIN, April 21, 2025 /PRNewswire/ -- Jazz Pharmaceuticals plc (Nasdaq: JAZZ) ("Jazz" or the "Company") today announced the successful completion of its acquisition of Chimerix, Inc. ("Chimerix") for approximately $935 million in cash. Chimerix is now a wholly owned subsidiary of Jazz. "Bringing Chimerix into Jazz adds a novel medicine to our oncology portfolio and advances our efforts to address unmet patient needs," said Bruce Cozadd, chairman and chief executive officer of Jazz. "Dordaviprone has the potential to become the first and only FDA-approved therapy for patients with H3 K27M-mutant diffuse glioma and offers a promising near-term commercial opportunity, if approved. We are excited to welcome Chimerix's talented team as we collectively continue to advance dordaviprone, leveraging our development and commercial capabilities to deliver this therapy to patients as soon as the second half of this year." The addition of dordaviprone, a novel first-in-class small molecule treatment in development for H3 K27M-mutant diffuse glioma, further diversifies and adds near-term commercial opportunity to Jazz's oncology pipeline. Dordaviprone is currently under Priority Review by the U.S. Food and Drug Administration (FDA), with a Prescription Drug User Fee Act (PDUFA) action date set for August 18, 2025. If approved in the U.S., the therapy may be eligible for a Rare Pediatric Disease Priority Review Voucher (PRV). Dordaviprone is also being studied in the ongoing Phase 3 ACTION trial to evaluate its use in newly diagnosed, non-recurrent H3 K27M-mutant diffuse glioma patients following radiation treatment, potentially extending its use into the first-line setting. Transaction Details Jazz's tender offer for all outstanding shares of common stock, par value $0.001 per share, of Chimerix expired at one minute after 11:59 p.m., Eastern Time, on April 17, 2025. Jazz has accepted for payment of $8.55 per share, in cash, without interest and subject to reduction for any applicable withholding taxes, all shares that were validly tendered and not validly withdrawn. Following its acceptance of the tendered shares, Jazz completed the acquisition of Chimerix through the merger of Pinetree Acquisition Sub, Inc., a Delaware corporation, an indirect wholly owned subsidiary of Jazz ("Purchaser") with and into Chimerix (the "Merger"). As a result of the Merger, the separate existence of Purchaser ceased, and Chimerix continued as the surviving corporation and an indirect wholly owned subsidiary of Jazz. Additional details regarding the tender can be found in a form 8-K filed by Jazz today with the SEC. About Dordaviprone Dordaviprone (ONC201) is a novel first-in-class small molecule imipridone that selectively targets the mitochondrial protease ClpP and dopamine receptor D2 (DRD2). Dordaviprone's unique mechanism of action includes alterations of key epigenetic modifications such as reversal of H3 K27me3-loss, which is the hallmark of H3 K27M-mutant gliomas. About Jazz Pharmaceuticals Jazz Pharmaceuticals plc (NASDAQ: JAZZ) is a global biopharmaceutical company whose purpose is to innovate to transform the lives of patients and their families. We are dedicated to developing life-changing medicines for people with serious diseases — often with limited or no therapeutic options. We have a diverse portfolio of marketed medicines, including leading therapies for sleep disorders and epilepsy, and a growing portfolio of cancer treatments. Our patient-focused and science-driven approach powers pioneering research and development advancements across our robust pipeline of innovative therapeutics in oncology and neuroscience. Jazz is headquartered in Dublin, Ireland with research and development laboratories, manufacturing facilities and employees in multiple countries committed to serving patients worldwide. Please visit www.jazzpharmaceuticals.com for more information. Caution Concerning Forward-Looking Statements This communication contains forward-looking statements that involve risks and uncertainties relating to future events and the future performance of Jazz and Chimerix, including statements regarding the prospective benefits of the acquisition, including benefits from dordaviprone's potential to become the first and only FDA-approved therapy for patients with H3 K27M-mutant diffuse glioma and its potential to offer a promising near-term commercial opportunity; the potential for a near-term commercial launch of dordaviprone in the U.S. if approved; the potential of the ongoing Phase 3 ACTION trial to confirm clinical benefit of dordaviprone in recurrent H3 K27M-mutant diffuse glioma and extend its use in first-line patients; dordaviprone potentially being eligible for a Rare Pediatric Disease PRV; and other statements that are not historical facts. Actual results could differ materially from those anticipated in these forward-looking statements. Risks that may cause these forward-looking statements to be inaccurate include, without limitation: uncertainties as to the difficulty of predicting the timing or outcome of regulatory approvals or actions, if any; risks related to the ability to realize the anticipated benefits of the acquisition, including the possibility that the expected benefits from the acquisition will not be realized or will not be realized within the expected time period and that Jazz and Chimerix will not be integrated successfully or that such integration may be more difficult, time-consuming or costly than expected; the effects of the transaction on relationships with employees, customers, suppliers, other business partners or governmental entities; significant transaction costs; unknown or inestimable liabilities; the risk of litigation and/or regulatory actions related to the acquisition; effectively launching and commercializing products and product candidates such as dordaviprone, if approved; the successful completion of development and regulatory activities with respect to dordaviprone; obtaining and maintaining adequate coverage and reimbursement for Jazz's products; the time-consuming and uncertain regulatory approval process, including the risk that Chimerix's NDA for dordaviprone seeking accelerated approval for treatment of H3 K27M-mutant diffuse glioma in adult and pediatric patients with progressive disease following prior therapy may not be approved by FDA in a timely manner or at all, and that Chimerix and/or Jazz may not receive a Rare Pediatric Disease PRV upon potential approval of dordaviprone; the costly and time-consuming pharmaceutical product development and the uncertainty of clinical success, including risks related to failure or delays in successfully initiating or completing clinical trials and assessing patients, including with respect to current and planned future clinical trials of dordaviprone; global economic, financial, and healthcare system disruptions and the current and potential future negative impacts to Jazz's business operations and financial results; and other risks and uncertainties affecting Jazz, including those described from time to time under the caption "Risk Factors" and elsewhere in its filings and reports with the U.S. Securities and Exchange Commission, including Jazz's Annual Report on Form 10-K for the fiscal year ended December 31, 2024 and future filings and reports. Other risks and uncertainties of which the Company is not currently aware may also affect its forward-looking statements and may cause actual results and the timing of events to differ materially from those anticipated. The forward-looking statements made in this press release are made only as of the date hereof, even if they are subsequently made available by the Company on its website or otherwise. The Company undertakes no obligation to update or supplement any forward-looking statements to reflect actual results, new information, future events, changes in its expectations or other circumstances that exist after the date as of which the forward-looking statements were made. Contacts Investors: Jeff Macdonald Executive Director, Investor Relations Jazz Pharmaceuticals plc InvestorInfo@jazzpharma.com Ireland +353 1 634 3211 U.S. +1 650 496 2717 Media: Kristin Bhavnani Head of Global Corporate Communications Jazz Pharmaceuticals plc CorporateAffairsMediaInfo@jazzpharma.com Ireland +353 1 637 2141 U.S. +1 215 867 4948 View original content to download multimedia:https://www.prnewswire.com/news-releases/jazz-pharmaceuticals-completes-acquisition-of-chimerix-302433612.html SOURCE Jazz Pharmaceuticals plc

Chimerix engaged with numerous biopharmas—termed parties A through G—about potential strategic transactions related to its oncology candidate.\n Before Jazz Pharmaceuticals inked a $935 million deal for Chimerix earlier this month, the biotech had been in talks with six other pharmas and one biotech about several different potential offers.Ultimately, Chimerix’s board went with Jazz’s offer due to its cash payable nature and the recent downward trend in biopharma financial markets, plus a general volatility tied to escalating political and global trade tensions, according to Securities and Exchange Commission documents filed Friday. Buying Chimerix gives Jazz control of the brain cancer drug candidate dordaviprone, also known as ONC201, a small molecule designed to treat recurrent H3 K27M-mutant diffuse glioma.But Jazz wasn’t first on site. Since August 2023, Chimerix had been in discussions about potential licensing deals for ONC201 with a “global pharmaceutical company,” known as “party A” in the filings. Chimerix steadily entered discussions with more companies—termed parties B through G—about potential strategic transactions related to the oncology candidate, per the docs.Jazz was one of the last contenders to enter the scene, reaching out after Chimerix publicly announced on Dec. 9, 2024, its plan to submit a new drug application seeking accelerated approval for ONC201 before the year ended.  On Dec. 18 and Dec. 19, Chimerix’s board reviewed its offers from several different companies, including a potential equity financing, a possible ex-U.S. licensing transaction for ONC201 and a potential acquisition. The board ultimately decided to move forward with multiple options at the same time. On the same day—Dec. 19—party A submitted a proposal to acquire ONC201 and related assets for an upfront payment of $350 million and a contingent payment of $100 million upon FDA approval. Chimerix management reached out to the pharma and said the proposal was insufficient.Less than a month later, party A tried again, submitting a new proposal to acquire Chimerix for an upfront payment of $6.50 per share and one contingent value right (CVR) per share, representing the right to receive a one-time payment of $1.50 upon FDA approval of ONC201 no later than June 2027, plus a one-time payment of $1.50 once ONC201 achieved yearly net sales in the U.S. above $700 million at a date before 2030.According to the filing, Chimerix’s board directed management to discuss transaction terms with party A in a way designed to increase the economic value and certainty of that proposal, with the view that alternative offers from other potential counterparties could be increased if they had a better nonbinding indication from party A.Meanwhile, bidders started dropping. Party F lost interest after Chimerix told the company that it had received a proposal with a higher value than party F’s offer of around $5 per share. And party G—a biotech—discontinued conversations regarding a potential acquisition on the belief that it wouldn’t be able to compete with other interested parties.  On Jan. 26, party A came back again with a revised proposal to acquire Chimerix on the same terms as before but with the deadlines to hit the payment-triggering milestones removed.Chimerix management told party A on Jan. 30 that the revised offer was still not good enough.On Feb. 6, Jazz outlined an acquisition offer for an upfront payment of $5.50 per share and one CVR per share representing the right to receive one-time payments of $1 upon FDA approval, 25 cents upon receipt of pricing approval in at least three major European markets and $1.50 once global net sales of ONC201 were above $500 million in a calendar year.Chimerix told Jazz the offer was insufficient but that Chimerix would provide additional due diligence materials so Jazz could increase its offer.Ten days later, Chimerix publicly announced that the FDA had accepted its new drug application seeking accelerated approval, with the application receiving priority review and a decision expected by Aug. 18 of this year.On March 3, Jazz submitted its final proposal: $8.55 per share in all cash. On the same day, party A shared a proposal to acquire Chimerix for an upfront payment of $7.50 per share and one CVR per share representing the right to receive a one-time payment of $2, payable if ONC201 received full approval by the FDA before Aug. 31, 2032. The CVR would not be payable if the primary endpoint of Chimerix’s ongoing phase 3 study was not achieved or if Chimerix received a complete response letter.That night, the board met to review the two offers and determined Jazz to be the winning bidder. The next afternoon, Chimerix’s board unanimously decided that Jazz’s offer was in the best interests of its stockholders. The board took into account several different factors when reaching its recommendation that stockholders accept the proposal, according to the documents.One consideration was that Jazz’s offer price represented a 70% premium over Chimerix’s closing price per share on March 3 and an 89% premium over the weighted average price of shares during the preceding 30-day trading period.The biotech board also considered the fact that the offer was payable fully in cash, allowing stockholders to realize immediate value.Furthermore, they weighed the risks of remaining a standalone company and general risks tied to market conditions. Among the potential risks identified were “the challenges faced by the biopharmaceutical industry,” including potential competition; “complex regulatory and political regimes”; and an “evolving pricing environment,” particularly in light of “the increasing scrutiny of pharmaceutical pricing and proposals to address the perceived high cost of pharmaceuticals.”Another consideration was “the current state of the U.S. and global economies, including the recent downward trend in the biopharmaceutical financial markets, U.S. trade policies and tariffs.” The board also cited rising inflation and volatility resulting from escalating political and global trade tensions in its ultimate decision.

Chimerix began attracting deal interest in its experimental brain tumor treatment about 18 months before it signed a $935 million cash exit to Jazz Pharmaceuticals earlier this month, according to a Friday regulatory filing . The filing highlights the broad interest that Chimerix was able to garner for dordaviprone, a potential first-ever medicine for H3 K27M-mutant diffuse glioma. Chimerix nabbed the asset as part of its Oncoceutics acquisition in 2021. The small molecule, also known as ONC201, is up for an FDA approval decision by Aug. 18. An undisclosed global biopharma company approached Chimerix in August 2023 about a potential ex-US licensing deal for the investigational drug. That initial confidentiality agreement with “Party A” kickstarted a year and a half of discussions between the North Carolina biotech and more than half a dozen other unnamed drugmakers interested in dordaviprone, according to the Friday filing. Jazz ultimately won with its $8.55 per-share cash offer. Jazz’s approach began in December 2024 , when its head of corporate development reached out to Chimerix a few days after the biotech said it was seeking accelerated approval of dordaviprone. A week later, on Dec. 19, Party A said it would be willing to acquire dordaviprone and related assets for $350 million upfront plus an additional $100 million if the FDA approved the medicine. The deal terms weren’t good enough, Chimerix replied. A month later, Party A came back and said it would be willing to acquire all of Chimerix for $6.50 per share, plus contingent payments if the biotech reached certain FDA and sales milestones. Meanwhile, Party F asked whether a $5 per-share offer was “attractive.” Chimerix’s financial advisor, Centerview, told the company it had already gotten a higher offer from another bidder. Over the next few weeks, Parties F, E and others dropped out of the discussions. Party F cited “perceived risks around Chimerix’s ongoing Phase 3 ACTION Study,” and Party E cited its “challenges in assessing risk in neuro-oncology,” according to Friday’s regulatory document. For its part, Jazz’s interest began at $5.50 per share on Feb. 6, plus various contingent value rights upon FDA approval, European reimbursement approvals and net sales milestones. Then, on Feb. 18, Chimerix said the FDA accepted its NDA with priority review status. Chimerix CEO Mike Andriole had already telephoned that message to Jazz CEO Bruce Cozadd and a Party A “senior executive” four days before. Weeks later, Jazz revised its proposal to $8.55 per share in cash, removing the riskiness of contingent value rights, which sometimes don’t come to fruition for acquired companies’ shareholders. On that same day, March 3, Party A said it would offer $7.50 per share upfront and a $2 per-share CVR if Chimerix snagged full FDA approval for dordaviprone by Aug. 31, 2032. But the CVR wouldn’t be payable if the Phase 3 trial failed or if Chimerix got a complete response letter from the FDA. In the end, Jazz beat Party A.