Tg(Mpz*Ala221fs)4Msch

The transgenic construct contains a mutant form of the mouse peripheral myelin protein zero (Mpz) gene in which a 97 bp fragment of a human MPZ cDNA, extending from the second nucleotide of exon 6 and encompassing the "P0ins" insertion associated with Dejerine-Sottas syndrome (DSS), has replaced the corresponding region of the mouse gene. The mutation comprises the insertion of CG between nucleotides 662 and 663, in exon 6, that causes a frameshift following amino acid 221 (Ala221fs). (J:82809)