Journal: Pediatric Nephrology (Berlin, Germany)
Article Title: Complement dysregulation associated with a genetic variant in factor H-related protein 5 in atypical hemolytic uremic syndrome
doi: 10.1007/s00467-023-06184-6
Figure Lengend Snippet: Factor H-related protein 5 wild-type and mutant proteins in cell supernatants and lysates. Factor H-related protein 5 was assayed by immunoblotting in transfected cell supernatants and lysates containing the wild-type sequence and the mutant M514R variant. Wild-type supernatant exhibited a strong band corresponding to FHR5. The supernatant from cells transfected with the CFHR5 M514R variant exhibited a very weak band. Control supernatants from HEK cells that were not transfected did not exhibit a band corresponding to FHR5. The FHR5 band was stronger in the lysates of the cells containing the M514R mutant variant compared to the wild-type, as the mutant variant was minimally secreted. hFHR5, recombinant human factor H-related protein 5; WT, wild-type; M514R, transfected mutant variant
Article Snippet: Recombinant hFHR5 was used as the standard (catalog number: 3845-F5, R&D Systems).
Techniques: Mutagenesis, Western Blot, Transfection, Sequencing, Variant Assay, Control, Recombinant