The mouse scurfy (sf) mutation is tightly linked to Gata1 and Tfe3 on the proximal X chromosome

In the mouse, the X-linked recessive mutation scurfy (sf) results in a rapidly fatal lymphoreticular disease that is characterized by runting, exfoliative dermatitis, lymphadenopathy, hepatosplenomegaly, thymic atrophy, and severe anemia. T cells mediate disease in the scurfy mouse, with CD4 super(+)CD8 super(-) T lymphocytes being the critical effector cells in disease pathogenesis. With both repulsion and coupling crosses, it had been demonstrated that the scurfy mutation maps near the centromere of the mouse X Chromosome (Chr) within two centimorgans (cM) of sparse fur (spf), a known point mutation within the ornithine transcarbamylase gene (Otc). In order to map sf relative to other loci on the proximal X Chr, we generated an interspecific Mus musculus/Mus spretus backcross segregating the sf mutation.