FIGURE 1.

Targeting vectors and resulting effects on fibrillin-1 in germ line mutant mice. A, in the GT-8 targeting vector (top), a poly(A) tail, eGFP, and a neomycin selection cassette (PGK-Neo, flanked by FRT sites) were placed in the intron between exons 34 and 35. A polyglycine linker (GGGG) and exon 33 splice acceptor site were engineered 5′ to the inverted coding region for eGFP. Cre-mediated recombination of special lox sites (lox66 and lox77) resulted in the inversion of eGFP behind exon 32, separated from exon 32 by the polyglycine linker. The neomycin selection cassette was removed after breeding to Flpe transgenic mice. In the H1Δ targeting vector (bottom), exon 7 is flanked by loxP sites. Cre-mediated recombination of the loxP sites results in deletion of exon 7 and the neomycin selection cassette. B, domain modules contained within the truncated GT-8 fibrillin-1 and within H1Δ fibrillin-1 are depicted, with wild-type fibrillin-1 shown for comparison.