Factor I is required for the development of membranoproliferative glomerulonephritis in factor H–deficient mice
J. Clin. Invest. Kirsten L. Rose, et al. 118:608 doi:10.1172/JCI32525 [Go to this article.]

Figure 1
Generation of Cfi^–/– mice. (A) Targeted Cfi locus, targeting vector, and structure of the targeted gene. Exons are represented by filled boxes, and the neomycin resistance gene (Neo) and herpes simplex virus thymidine kinase cassette (HSV-tk) are indicated. Homologous fragments are indicated by dotted lines. E, EcoRI. Double-headed arrows indicate the fragments detected on Southern blot analysis of wild-type and recombinant alleles after EcoRI digestion of genomic DNA hybridized with the 3′ probe (P1, a cDNA probe containing exons 5 and 6). (B) Amplification of recombinant and wild-type alleles using genomic DNA from Cfi^–/–, Cfi^+/–, and wild-type mice. Absence of the wild-type allele is seen in the Cfi^–/– animal. (C) Western blot using nonreducing conditions for serum factor I using crossreactive polyclonal anti-human factor I anti-sera. Lane 1 indicates normal human sera, lane 2 indicates sera from a factor I–deficient individual. Lanes 3–8 represent mouse sera from homozygous, heterozygous, and wild-type mice, as indicated. Absence of the 88-kDa factor I protein (box) is evident in the factor I–deficient individual (lane 2) and in sera from the Cfi^–/– mice (lanes 3 and 4).