Undisclosed factor H therapeutic / Eleva - LARVOL DELTA

The 4 functional segments of Factor H: Role in physiological target recognition and contribution to disease. (PubMed, J Immunol) - "Factor H function is modulated by Factor H like protein 1 and FHR1, 2 plasma proteins that share segments with Factor H. This interplay is critical for fine-tuning local complement. Understanding Factor H's physiological role, as well as the impact of its absence, mutations, or autoantibody targeting, provides insights into disease mechanisms and provides opportunities for therapeutic intervention by using full-length Factor H, its fragments, or complement-modulatory compounds."

Journal • Age-related Macular Degeneration • ANCA Vasculitis • Atypical Hemolytic Uremic Syndrome • Complement-mediated Rare Disorders • Glomerulonephritis • Macular Degeneration • Nephrology • Oncology • Ophthalmology • Retinal Disorders • Vasculitis

Moss-derived human complement factor H modulates retinal immune response and attenuates retinal degeneration. (PubMed, J Neuroinflammation) - "Modulating the alternative complement pathway using moss-derived recombinant human full-length CFH variant CPV-101 and CPV-104 counter-regulate gliosis and attenuates light-induced retinal degeneration, highlighting a promising concept for the treatment of AMD patients."

Journal • Age-related Macular Degeneration • Ophthalmology

Effective long-term treatment with moss-produced factor H by overcoming the antibody response in a mouse model of C3G. (PubMed, Front Immunol) - "Complement-associated disorders are caused by the dysregulation and disbalance of the complement system, especially excessive activation. Repeated CPV-104 administration was able to lastingly resolve C3 deposits, offering additional rationale for the clinical testing of CPV-104 in human C3G patients. Moreover, our novel dual-depletion method has the potential for adaptation to different mouse models, allowing the testing of multiple doses of other therapeutic proteins."

Journal • Preclinical • Complement-mediated Rare Disorders • Glomerulonephritis • CD20 • CD4

Moss-Produced Human Factor H (CPV-104): Pioneering Rebalancing Therapies for Kidney Complement Disorders (KIDNEY WEEK 2024) - "By optimizing dosing intervals and exploring s.c. administration, FH availability was significantly improved in conjunction with sustained reduction of glomerular C3 deposits in preclinical models. These findings provide valuable insights into the clinical translation of CPV-104 as a new therapeutic strategy for complement-mediated renal diseases, enabling physiological complement functionality."

Complement-mediated Rare Disorders • Renal Disease

Moss-produced human complement factor H with modified glycans has an extended half-life and improved biological activity. (PubMed, Front Immunol) - "Most drugs that target the complement system are designed to inhibit the complement pathway at either the proximal or terminal levels. Furthermore, CPV-104 showed comparable functionality to serum-derived FH in vitro, as well as similar performance in ex vivo assays involving samples from patients with atypical hemolytic uremic syndrome, C3 glomerulopathy and paroxysomal nocturnal hematuria. CPV-104 - the human FH analog expressed in moss - will therefore allow the treatment of complement-associated human diseases by rebalancing instead of inhibiting the complement cascade."

Journal • Atypical Hemolytic Uremic Syndrome • Complement-mediated Rare Disorders • Glomerulonephritis • Nephrology

An Interdisciplinary Diagnostic Approach to Guide Therapy in C3 Glomerulopathy. (PubMed, Front Immunol) - "Such a combined diagnostic approach was used here for a 42-year-old female patient with a novel mutation in the Factor H gene, C3 glomerulopathy and signs of chronic endothelial damage. We present here an approach that might in future help to guide therapy of renal diseases with relevant complement activation, especially since diverse new anti-complement agents are under clinical investigation."

Journal • Complement-mediated Rare Disorders • Glomerulonephritis • Immunology • Lupus Nephritis • Nephrology • Renal Disease