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Nucleotide substitutions (TGG to TAG) were introduced into exon 9 that result in the amino acid substitution of tryptophan with a stop at position 392 (W392X). A floxed neo cassette inserted between exons 8 and 9 was removed by cre mediated recombination leaving a single loxP site. The mutation mimicks one identified in patients with mucopolysaccharidosis type I-Hurler (MPS I-H), also known as Hurler syndrome. RT-PCR confirmed a 30% to 50% reduction in steady-state transcript levels. (J:155619)