Production of Normal ↔ Macular Mouse Chimeras: The Presence of Critical Tissue in the Macular Mutant Mouse, a Model of Menkes' kinky Hair Disease

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The macular mouse (Mo^<ml>) is a murine model of the Menkes' kinky hair disease. The Mo^<ml> allele is a recessive lethal mutation. Mo^<ml> mice die about 15 days after birth, exhibiting behavior of some neurological disorders without evidence of a connective tissue abnormality. A multitude of morphologic abnormalities have been described in hemizygotes (Mo^<ml>/Y) and homozygotes (Mo^<ml>/Mo^<ml>). As an approach to determine the critical tissue in the macular mutant mouse, we produced chimeras from macular mutant and normal mouse embryos. Thirteen chimeras were obtained, and 11 of these chimeras survived without copper treatment. In surviving chimeras, normal mouse cells were present in all tissues. However, 2 chimeras died at days 16 and 22 after birth, respectively. The normal mouse cells were not distributed in the liver of both chimeras. The copper concentration in the liver of both chimeras were significantly low in the same manner as the Mo^<ml>/Y and Mo^<ml>/Mo^<ml> mouse. Our findings provide important evidence in the search for "critical tissue" in the macular mouse.
社団法人日本動物学会の論文
1993-08-16