Unique Target Binding by the C-Terminal Region of FHR1 Provides a New Perception of aHUS Pathology

Luce Perie, Selina Stippa, Christoph Saffer B, Andrea Hartmann, Mathias Mörgelin, Svante L. H. Zipfel, Marc Thilo Figge, Thorsten Wiech, Christine Skerka, and Peter F. Zipfel (2024). Unique Target Binding by the C-Terminal Region of FHR1 Provides a New Perception of aHUS Pathology. Frontiers in Hematology.

Factor H-related protein 1 (FHR1) is implicated in atypical hemolytic uremic syndrome (aHUS), a rare disease of complement dysregulation. This work models the domain geometry of FHR1’s C-terminal region and quantifies its distinct target-binding behavior from assay data, contributing a revised structural and mechanistic picture of how it drives aHUS pathology.