CCN4

NORTH CHICAGO, Ill., May 1, 2025 /PRNewswire/ -- BLR Bio, a biotechnology company in Rosalind Franklin University's Helix 51 incubator, announced new data on its novel therapy for systemic sclerosis and lung fibrosis at the British Society for Rheumatology (BSR) Annual Conference this week in Manchester, UK. BLR Bio CEO and CSO Bruce Riser, a renowned expert in fibrotic diseases, pictured in his lab at Rosalind Franklin University's Helix 51 biomedical incubator. Interstitial Lung Disease (ILD) is a devastating disorder, encompassing a group of chronic lung conditions that affect the tissue between the air sacs in the lungs. ILD conditions can include Idiopathic pulmonary fibrosis (IPF), which can result from systemic sclerosis. The estimated prevalence in the United States is 654,852, according to a February 2024 publication in the journal CHEST. "These patients are in urgent need of a therapy that can halt or reverse this disease without the threat of serious side effects," said BLR Bio CEO and CSO Dr. Bruce Riser. In the new study, presented by Dr. Andrew Leask at the University of Saskatchewan in Canada, BLR 200, the company's lead compound, demonstrated the ability to reduce key disease tissue markers for fibrotic lung disease. Reductions were shown in Ashcroft scores, collagen levels, and both fibronectin-1 (FN-1) and profibrotic CCN4, signaling molecules involved in the development of scar tissue. Dr. Leask is a leading expert in fibrosis and CCN proteins and a long-term collaborator with BLR Bio. "Our data shows the reductions in Ashcroft scores and collagen levels appear to be more pronounced than those seen with other therapies on the market and in development," Dr. Riser said. "Combined with prior observations modeling scleroderma skin fibrosis, BLR-200 has shown the potential to address both skin and lung fibrosis. It appears to be the only drug in development that reduces expression of all three CCN proteins — CCN1, CCN2, CCN4 — that play a crucial role in the activation of fibroblast cells critical for initiating and maintaining fibrosis." Dr. Joseph DiMario, RFU Interim VP for Research, noted that BLR Bio was one of the first tenants of Helix 51. "We are very pleased to see the progress the company is making toward the development of life-changing medicines," he said. About Rosalind Franklin University Rosalind Franklin University of Medicine and Science embodies the spirit of inquiry and excellence modeled by its namesake Dr. Rosalind Franklin, whose Photo 51 was crucial to solving the structure of DNA. Recognized for its research in areas including neuroscience, brain-related diseases, inherited disorders, diabetes, obesity, and gait and balance, RFU encompasses the Chicago Medical School, College of Health Professions, College of Nursing, College of Pharmacy, School of Graduate and Postdoctoral Studies and the Dr. William M. Scholl College of Podiatric Medicine. Learn more at rosalindfranklin.edu. About BLR Bio Learn more at blrbio.com. Office of Marketing and Communications [email protected] SOURCE Rosalind Franklin University of Medicine and Science WANT YOUR COMPANY'S NEWS FEATURED ON PRNEWSWIRE.COM? 440k+ Newsrooms & Influencers 9k+ Digital Media Outlets 270k+ Journalists Opted In GET STARTED

Mediar to advance MTX-463 through an IPF Phase 2 trial, with expected initiation in 1H 2025 BOSTON, MA, USA I January 10, 2025 I Mediar Therapeutics, Inc. , a clinical stage biotechnology company advancing a portfolio of first-in-class therapies designed to halt fibrosis progression, announced a global licensing agreement with Eli Lilly and Company to advance MTX-463 into a Phase 2 clinical trial for idiopathic pulmonary fibrosis (IPF). MTX-463 is a first-in-class human IgG1 antibody designed to neutralize WISP1-mediated fibrotic signaling in several debilitating diseases. The Phase 1 study was recently completed in healthy volunteers and showed MTX-463 to be well-tolerated and engaged WISP1 at all tested doses. The Phase 2 IPF study is designed to evaluate safety, pharmacokinetics, and efficacy in patients. The trial is expected to initiate in the first half of 2025 and will be conducted by Mediar. Following completion of the Phase 2 study, Lilly will have the right to lead all further clinical development and commercialization of the program. “This collaboration supports our unique myofibroblast-directed approach to treating fibrotic diseases and our mission to bring first-in-class therapies to patients with high unmet medical need,” said Rahul Ballal, Ph.D., Chief Executive Officer of Mediar Therapeutics. “By combining Lilly’s unparalleled expertise in bringing life-changing medicines to patients with our novel scientific approaches, we are excited to advance a robust Phase 2 IPF program and potentially bring new therapies that halt fibrosis.” Under the terms of the agreement Mediar will receive a combined $99 million, which is inclusive of an upfront payment and near-term milestones. Mediar may receive up to an additional $687 million in potential downstream development and commercialization milestones. Additionally, Mediar is eligible to receive high-single to low-double digit royalty payments and net sales milestones based on potential future product sales. “Mediar’s scientific approach and experienced team has led to the creation of novel, potential first-in-class therapies for fibrotic diseases, including MTX-463,” said Mark Genovese, M.D., senior vice president of Lilly Immunology development. “The collaboration with Mediar exemplifies our dedication to fostering innovation, and we look forward to partnering with the Mediar team to advance MTX-463 through development in hopes of bringing a novel treatment option to people living with IPF.” Beyond MTX-463, Mediar will continue to independently advance its two wholly owned programs to treat fibrotic disorders. MTX-474 is a first-in-class human IgG1 antibody designed to neutralize the EphrinB2 signaling that causes fibrosis, and is currently finishing a Phase 1 clinical study. Mediar anticipates initiating a Phase 2 trial for MTX-474 in systemic sclerosis in the second half of 2025. Mediar’s third novel fibrosis program, targeting SMOC2, is also advancing with a plan to nominate a clinical candidate in the first half of 2025. About MTX-463 MTX-463 is a first-in-class human IgG1 antibody developed against WNT1-inducible signaling pathway protein-1 (WISP1). WISP1 is a secreted matricellular protein shown to have a relevant role in fibrosis progression, measurable in human blood, and correlates with disease severity. Initial data indicates that MTX-463 neutralizes WISP1-mediated fibrotic signaling that spans several fibrotic indications and significantly reduced fibrosis in vitro and in preclinical mouse models. Mediar expects to initiate a Phase 2 study in IPF with MTX-463 in 1H-2025. About MTX-474 MTX-474 is a first-in-class human IgG1 antibody designed to neutralize the EphrinB2 signaling that causes the onset and progression of fibrosis. Ephrin ligands and Eph receptors mediate biological processes involved in tissue fibrosis including cell migration, myofibroblast activation, and tissue remodeling. A growing body of evidence has implicated EphrinB2 in the fibrosis of the skin, lungs, and heart. Expression of EphrinB2 and its receptors are measurable in human blood and correlates with disease severity. Mediar expects to initiate a Phase 2 study in SSc with MTX-474 in 2H-2025. About Mediar Therapeutics Mediar Therapeutics is pioneering a new approach to fibrosis treatment that aims to halt the disease at a different source – the myofibroblast, the key pathogenic cell in fibrosis that drives scarring, disease progression, and ultimately organ failure. Mediar was founded based on a deep understanding of the complex science underlying fibrosis progression. By combining novel targets with reliable, easily detectable blood biomarkers and familiar modalities, Mediar’s goal is to bring forward novel anti-fibrotic therapies that potentially have a precision medicine approach. For more information, contact info@mediartx.com or follow us on LinkedIn . SOURCE: Mediar Therapeutics

Eli Lilly is one of a handful of Big Pharmas that secured a close look at Mediar Therapeutics through venture investments. \n Eli Lilly is making a play for the idiopathic pulmonary fibrosis (IPF) market, offering Mediar Therapeutics $99 million in upfront and near-term payments for global rights to an asset that is entering phase 2.The deal, which includes up to $687 million in milestones, gives Lilly global rights to the anti-WISP1 antibody MTX-463. Researchers have linked WISP1 to fibrosis progression, leading Mediar to identify the protein as a way to reduce scarring in IPF and other diseases driven by the accumulation of scar tissue. Mediar recently finished a phase 1 trial in healthy volunteers.With the study showing MTX-463 is well tolerated and engages WISP1, Lilly has swooped in to secure an asset that could unlock a market currently dominated by Boehringer Ingelheim’s Ofev and Roche’s Esbriet. Efforts to create next-generation products have largely ended in failure, although Boehringer reported a phase 3 win last year.Lilly has been an observer as other companies have swung and missed at IPF, but it does have some links to the indication. Endeavor BioMedicines is testing an ex-Lilly molecule in IPF. Morphic once had an IPF collaboration with AbbVie, but the biotech was focused on inflammatory bowel disease by the time Lilly bought it last year. The Mediar deal establishes Lilly as a player in the IPF space, albeit one that is further from market than some of the other runners and riders. Mediar plans to start a phase 2 trial of MTX-463 in the first half of 2025. The biotech will run the midphase trial and then hand over to Lilly for further development.Lilly is one of a handful of Big Pharmas that secured a close look at Mediar through venture investments. Bristol Myers Squibb, Lilly, Novartis Venture Fund, Ono Venture Investment and Pfizer Ventures invested in 2023 in the $105 million financing that positioned Mediar to enter the clinic. The funding reflected the potential for Mediar to deliver a first-in-class therapy that more precisely treats IPF than existing drugs.