An FRT-flanked pgk-neo cassette was inserted upstream of exon 6 and a copy of exon 6 with a G to A mutation at position 547 resulting in an G to R substitution at amino acid 182 was inserted via homologous recombination. Flp mediated recombination removed the pgk-neo cassette. The mutation in exon 6 is homologous to the most common type of COL3A1 disease-causing genetic variant in human Vascular Ehlers-Danlos syndrome patients. (J:330320)