NI-006

Neurimmune today announced that the U.S. Food and Drug Administration (FDA) has granted Alexion, AstraZeneca Rare Disease Fast Track designation for the development of ALXN2220 (formerly NI006) for the treatment of transthyretin amyloidosis with cardiomyopathy (ATTR-CM). ATTR-CM is a systemic, progressive and life-threatening disease caused by the deposition of misfolded transthyretin (TTR), which can lead to heart failure. Despite advances in slowing disease progression, there is no available treatment option designed to deplete TTR from the heart to revert cardiac dysfunction. The Fast Track designation was granted based on the results of a Phase 1b trial conducted by Neurimmune and published in The New England Journal of Medicine in 2023.1 "In our Phase 1b trial intravenously administered ALXN2220 reduced cardiac tracer uptake and extracellular volume. The reduction of these surrogate markers of cardiac amyloid load suggests that ALXN2220 can induce the depletion of cardiac amyloid deposits by the immune system. This is a novel therapeutic mechanism with potential to restore cardiac function in patients suffering from ATTR amyloidosis,” said Christoph Hock, MD, Chief Medical Officer of Neurimmune. “We look forward to Alexion’s continued development of ALXN2220 and are very pleased the FDA’s Fast Track designation will help accelerate this effort.” ALXN2220 is an investigational human monoclonal antibody currently being evaluated in the Phase 3 DepleTTR-CM trial to assess its efficacy as an add-on treatment to the standard of care for patients with ATTR-CM. The primary endpoint is a composite of all-cause mortality and cumulative frequence of cardiovascular clinical events. The Fast Track designation is granted to expedite the review of drugs to treat serious conditions and address an unmet medical need. The designation allows for more frequent interactions with the FDA, and potentially in the future, Accelerated Approval and Priority Review in the U.S. In 2022, Neurimmune entered into an exclusive global collaboration and license agreement with Alexion, AstraZeneca Rare Disease for ALXN2220. Neurimmune is responsible for the completion of the Phase 1b clinical study on behalf of Alexion, with Alexion incurring certain trial costs. Aside from the Phase 1b trial, Alexion is responsible for further clinical development, manufacturing, and commercialization of ALXN2220. 1Garcia-Pavia et al., N Engl J Med 2023 Neurimmune is a biopharmaceutical company translating human immune memory into antibody therapeutics. Neurimmune develops drug candidates for CNS and related protein aggregation diseases including Alzheimer’s disease, amyotrophic lateral sclerosis, frontotemporal dementia and ATTR cardiomyopathy. With its Reverse Translational MedicineTM technology, Neurimmune discovered the brain amyloid depleter NI101SQ for Alzheimer’s disease, anti-SOD1 antibody AP-101 for ALS and the anti-ATTR antibody ALXN2220 (formerly NI006) for ATTR cardiomyopathy, programs being currently evaluated in clinical trials. John Capodanno (US) john.capodanno@dentonsglobaladvisors.com Martin Meier-Pfister (Switzerland) media@neurimmune.com

Neurimmune today announced that long-term safety and efficacy data from the open-label extension of a Phase I trial related to ALXN2220 (formerly NI006) for the treatment of transthyretin amyloid cardiomyopathy (ATTR-CM) will be presented in London at the ESC Congress 2024, a scientific congress of the European Society of Cardiology (ESC), on September 1, 17:48 UK time. Professor Peter van der Meer from the University Medical Centre Groningen, Netherlands, will present during the session for Advances in Amyloidosis. Presentation Details: Title: Long-term safety and efficacy of antibody ALXN2220 for depletion of cardiac amyloid transthyretin: Results of treatment beyond 12 months in the open-label extension of study NI006-101 Presenter: Peter van der Meer, MD, PhD, Cardiologist, University Medical Center Groningen (UMCG) Session: Advances in Amyloidosis Date: September 1, 2024 Time: 17:48 UK time Location: Science Box 1 Amyloid transthyretin cardiomyopathy (ATTR-CM) is increasingly recognized as a treatable cause of heart failure. Progressive ATTR amyloid depositions characterize the disease, leading to progressive heart failure and a high rate of fatality if left untreated. Despite recent advances in slowing disease progression, there is no treatment available to deplete cardiac ATTR amyloid and revert cardiac dysfunction. ALXN2220 is an investigational human monoclonal antibody designed to deplete amyloid deposits in ATTR-CM as add-on to standard of care.1,2 At the ESC Congress 2024, Neurimmune, Alexion, AstraZeneca Rare Disease and the clinical investigators will present new long-term safety, tolerability and efficacy data of ALXN2220 treatment beyond 12 months which was studied in a prolonged Open-Label Extension of the NI006-101 trial. Prof. Christoph Hock, Chief Medical Officer of Neurimmune, said: “The depletion of the cardiac ATTR amyloid deposits is a rational therapeutic target to revert disease pathology and restore organ function. Our data at ESC Congress 2024 underscores the commitment of Neurimmune and our partner Alexion to evaluate the safety and efficacy of longer-term ALXN2220 treatment to develop a best-in-class option for patients with ATTR-CM and to increase our understanding of the mechanisms underlying amyloid depletion.” In 2022, Neurimmune entered into an exclusive global collaboration and license agreement with Alexion, AstraZeneca Rare Disease, for ALXN2220. Neurimmune is responsible for completion of the Phase 1b clinical study on behalf of Alexion, with Alexion incurring certain trial costs. Aside from the Phase 1b trial, Alexion is responsible for further clinical development, manufacturing, and commercialization as ALXN2220. In 2024, Alexion initiated the Phase 3 DepleTTR-CM clinical study to assess the efficacy and safety of ALXN2220 for the treatment of ATTR-CM. 1Garcia-Pavia et al., N Engl J Med 2023; 389(3):239. 2Michalon et al., Nat Commun 2021; 12(1):3142. Neurimmune is a biopharmaceutical company translating human immune memory into antibody therapeutics. Neurimmune develops drug candidates for CNS and related protein aggregation diseases including Alzheimer’s disease, amyotrophic lateral sclerosis, frontotemporal dementia and ATTR cardiomyopathy. With its Reverse Translational MedicineTM technology, Neurimmune discovered the brain amyloid depleter NI101SQ for Alzheimer’s disease, anti-SOD1 antibody AP-101 for ALS and the anti-ATTR antibody ALXN2220 (formerly NI006) for ATTR cardiomyopathy, programs being currently evaluated in clinical trials. John Capodanno (US) john.capodanno@dentonsglobaladvisors.com Martin Meier-Pfister (Switzerland) media@neurimmune.com