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. 2020 Dec 7;32(1):99–114. doi: 10.1681/ASN.2020050698

Figure 1.

Figure 1.

Generation of C3hu/hu mice. (A) An illustration, not to scale, of the mouse C3, mouse C3 KO, and humanized C3 genomic loci. In two sequential targeting steps, the mouse C3 gene, including 6.6 kb of 5′ regulatory region and the entire coding region from exon 1 through exon 41, is deleted and replaced by the human C3 gene, including 9 kb of 5′ regulatory region, the entire coding region from exon 1 through exon 41, 1.5 kb of sequence downstream of the polyA signal, and a loxP site. Horizontal black lines indicate mouse sequence; the horizontal double gray line indicates human sequence; vertical black lines indicate exons; black boxes indicate 3′ untranslated regions; white arrows indicate loxP sites; sequence lengths in kilobases are indicated below the sequences. (B) Expression of the C3 gene in WT (n=3) and C3hu/hu (n=3) by next-generation sequencing (NGS). (C) Serum C3 levels in WT (n=8) and C3hu/hu (n=16). (D) Serum C5 levels in WT (n=7) and C3hu/hu (n=12). (E) Graph showing survival curve of WT (n=17) and C3hu/hu (n=137) mice. (F) Graph showing results of serum C3 levels postadministration of 1 mg CFH (n=3), 2 mg CFH (n=4), 4 mg CFH (n=3), CFI (n=2), or CFH and CFI (n=3) in C3hu/hu mice. ****P<0.001, versus WT. Chr17, chromosome 17; FPKM, fragments per kilobase of transcript per million mapped reads; pNHS, pooled normal human serum (BioIVT).