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Exon 6 and flanking non-coding sequences were replaced with a 2 kb neomycin resistance cassette via homologous recombination. The targeted mutation results in deletion of 101 C-terminal residues and disrupts enzyme function. Gene expression was absent in homozygous mutant animals in all tissues examined (lung, liver, kidney, colong, skeletal muscle) by Northern blot and Western blot. (J:46669)