Complement Factor H Related Protein 1 (CFHR1)

CFHR1 encodes a secreted protein belonging to the complement factor H protein family. It binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It is proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement, and facilitating tissue invasion. Mutations in this gene are associated with an increased risk of atypical hemolytic-uremic syndrome. Factor H, via SCR domains 6 to 7 and 19 to 20, and FHR1, via SCR domains 3 to 5, bound to surface-expressed Pseudomonas aeruginosa elongation factor Tuf and also to recombinant Tuf. Factor H and plasminogen (PLG) bound simultaneously to Tuf, and PLG was proteolytically activated.