A construct consisting of a wmN1 enhancer, a heat shock protein (Hsp) promoter, the human PLP1 cDNA modified c.89T-G (resulting in p.Leu30Arg) and a polyadenylation (PolyA) signal, plus an Hprt1 promoter and exon 1 was inserted at the 5' end of Hprt1 in reverse orientation in ES cells that carried a deletion of the Hprt1 promoter and exon 1. The recombined locus contains a restored Hprt1 gene and the expression construct in tandem reverse orientation to the Hprt1 gene. (J:238644)