NI009 (AL Amyloidosis)

AL amyloidosis is a rare, systemic and progressive disorder caused by defective plasma cells that produce misfolded immunoglobulin light chains. These misfolded light chains aggregate into insoluble amyloid fibrils that deposit in tissues and organs throughout the body. NI009 is a preclinical human monoclonal antibody designed to target and deplete light chain fibrils and deposits from affected tissues and organs in AL amyloidosis.

Aggregated Light Chains Cause Organ Damage and Dysfunction

In AL amyloidosis, abnormal kappa (κ) or lambda (λ) light chain proteins produced by these plasma cells misfold, aggregate and form amyloid fibrils that deposit in tissues and organs. Left untreated, the accumulation of these toxic amyloid deposits, particularly in the heart and kidneys, can cause progressive organ damage and dysfunction and may lead to premature death, most commonly due to cardiac failure.

NI009 Designed to Clear Aggregated Light Chains

By leveraging its expertise in designing therapeutics that trigger immune-mediated clearance of disease-causing protein aggregates, Neurimmune developed the novel antibody NI009 to deplete amyloid light chain deposits. NI009 exhibits broad activity against amyloids of diverse lambda light chain subtypes across patients despite the high clonal heterogeneity of the disease. The lead candidate NI009 is currently in advanced preclinical development.

Leveraging our expertise in designing therapeutics that harness immune-mediated clearance of pathogenic protein aggregates, we have developed a novel antibody with the potential to efficiently deplete amyloid light-chain deposits.

Roger M. Nitsch, CEO

Partnership with Alexion, AstraZeneca Rare Disease

In 2025, Neurimmune has expanded on its transthyretin amyloid cardiomyopathy (ATTR-CM) collaboration with Alexion, AstraZeneca Rare Disease. Within the collaboration, Neurimmune will continue to be responsible for preclinical development, initial manufacturing activities and conducting the first-in-human clinical study. Alexion will be responsible for further clinical development, manufacturing, and commercialization.