Ctr1flox floxed mutant mice possess loxP sites flanking exons 1-4 of the solute carrier family 31, member 1 (Slc31a1) gene. Slc31a1 encodes Copper (Cu) transporter 1 (CTR1), a high affinity Cu+ transporter shown to traffic from the plasma membrane to intracellular vesicles, regulating intestinal copper absorption. Mutations in Ctr1 have been associated with diseases characterized by defects in intestinal Cu absorption and Cu distribution in the liver, e.g., Menkes disease and Wilson disease. Mice that are homozygous for this allele are viable and fertile. When these mutant mice are bred to mice that express Cre recombinase, resulting offspring will have loxP-flanked sequences deleted in cre-expressing tissues.
For example, when crossed to B6.SJL-Tg(Vil-cre)997Gum/J mice
(Stock No. 004586) expressing Cre recombinase in intestinal epithelial cells, the resulting Ctr1int/int mice die by three weeks of age and display hypopigmentation, skin laxity, ataxia, and brittle, kinky whiskers, as seen in patients with the human Cu+ homeostasis disease, Menkes kinky hair disease. They have elevated Cu+ levels in intestinal epithelial cells and liver, and decreased levels in heart, brain, kidney and spleen.
