A recessive mutation exhibiting severe myelin breakdown, mainly at the level of the lumbar segments of the spinal cord and without any associated inflammation, was discovered in a partially inbred rat colony. Analysis of the segregation patterns of a set of polymorphic microsatellite markers in two inter-strain crosses allowed the mapping of this autosomal recessive mutation to rat Chromosome (Chr) 17, very close to the prolactin (Prl) locus, in a region homologous to human Chr 6p21.2-22.3 and mouse Chr 13. The pathology of the demyelination process and the chromosomal localization indicate that this mutation has no known equivalent in either mouse or human.