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. 2014 Jun 5;26(1):209–219. doi: 10.1681/ASN.2013121339

Figure 5.

Figure 5.

The FHR1/FH hybrid protein. (A) The protein product of the CFHR1/CFH hybrid gene is a five-SCR protein, where SCR1, -2, -3, and -4 are derived from FHR1 and SCR5 is from FH. (B) Western blot of the FHR1/FH hybrid protein was performed using a polyclonal goat anti-FH antibody against sera from the proband with the heterozygous duplication and the hybrid CFHR1/CFH gene (II:1), his wife (II:2) with two normal copies of CFHR1, a control with two copies of CFHR1 (Ctr 1), two controls with one copy of CFHR1 (Ctr 2 and Ctr 3), and a control with zero copies of CFHR1 (Ctr 4). The presence of the FHR1/FH hybrid protein accounts for the increased band density seen in the proband (II:1). (C) Western blot of the FHR1/FH hybrid protein was performed using a mouse anti–FHR1-FHR2-FHR5 mAb against sera from the proband (II:1) and his wife (II:2). The densities of the two bands of FHR1 isoforms in the proband serum are more pronounced than the densities in his wife’s serum, suggesting the presence of secreted FHR1/FH hybrid protein.