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Factor-H-related protein 1 (FHR1), a promotor of para-inflammation in age-related macular degeneration
Journal article   Open access   Peer reviewed

Factor-H-related protein 1 (FHR1), a promotor of para-inflammation in age-related macular degeneration

Andjela Sekulic, Sarah M Herr, Kelly Mulfaul, Inga-Marie Pompös, Silvia Winkler, Carola Dietrich, Benedikt Obermayer, Robert F Mullins, Thomas Conrad, Peter F Zipfel, …
Journal of neuroinflammation, Vol.22(1), 173
07/03/2025
DOI: 10.1186/s12974-025-03499-z
PMCID: PMC12226897
PMID: 40611130
url
https://doi.org/10.1186/s12974-025-03499-zView
Published (Version of record) Open Access

Abstract

Age-related macular degeneration (AMD), a multifactorial type of retinal degeneration represents the most common cause for blindness in elderly. Polymorphisms in complement factor-H increase, while absence of factor-H-related protein-1 (FHR1) decreases the AMD risk, currently explained by their opposing relationship. Here we identify a FHR1-driven pathway fostering chronic cellular inflammation. FHR1 accumulates below the retinal pigment epithelium (RPE) in AMD donor tissue and similarly the murine homolog, muFHR1 is abundant in three AMD-relevant mouse models. These mouse models express the muFHR1 receptor EGF-like module-containing mucin-like hormone receptor 1 (Emr1) on the RPE and on invading mononuclear phagocytes (MP), where both cells form clusters via muFHR1/Emr1. FHR1 ignited EMR2-dependent Ca -signals and gene expression in both human RPE cell line and in vivo where muFHR1 affects Emr1 cells (RPE and MP) gene expression shown by RNAseq analysis. As muFHR1 deletion in mice revealed significantly reduced MP invasion and neoangiogenesis in laser-induced choroidal neovascularization, we hypothesize that FHR1 accumulates, stabilizes and activates MP in the stage of RPE degeneration.
Animals Complement C3b Inactivator Proteins - genetics Complement C3b Inactivator Proteins - metabolism Disease Models, Animal Humans Inflammation - genetics Inflammation - metabolism Inflammation - pathology Macular Degeneration - genetics Macular Degeneration - metabolism Macular Degeneration - pathology Mice Mice, Inbred C57BL Mice, Knockout Mice, Transgenic Retinal Pigment Epithelium - metabolism Retinal Pigment Epithelium - pathology

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