These Cp knock-out mice exhibit a progressive accumulation of iron in hepatocytes and reticuloendothelial cells.
Dr. Zena L. Harris, Johns Hopkins Univ School of Medicine
Mice that are homozygous null for the Cp gene demonstrate a progressive accumulation of iron in hepatocytes and reticuloendothelial cells. By one year of age, iron content of the liver and spleen reaches three to six-fold that observed in wild-type litter mates. The Cp gene product appears to be necessary for proper iron efflux from these cell types. This strain represents a viable murine model for aceruloplasminemia. At one year of age there is no evidence of anemia, diabetes, or neurological symptoms despite iron accumulation at the corresponding tissue sites.
The majority of exon 17 and exon 18 has been replaced with a neomycin cassette resulting in the generation of an unstable mRNA. The initial chimeric male was bred with a Black Swiss Webster female. This strain originated on a B6,129 background and is maintained as a heterozygote. Subsequent matings were heterozygous intercrosses.
|Allele Name||targeted mutation 1, Z Leah Harris|
|Allele Type||Targeted (Null/Knockout)|
|Allele Synonym(s)||Cp-; CpKO|
|Gene Symbol and Name||Cp, ceruloplasmin|
|Strain of Origin||129X1/SvJ|
|Molecular Note||A neomycin resistance cassette replaced a genomic fragment containing part of exon 17, intron 17 and part of exon 18, which include sequences encoding the residues essential for the formation of the trinuclear copper cluster of the encoded protein. Western blot analysis on serum derived from homozygous mice demonstrated that no detectable encoded protein was present, and activity assays on serum of homozygous mice confirmed that no functional protein is made from this allele.|
|Mutations Made By|| |
Dr. Zena Harris, Johns Hopkins Univ School of Medicine
When maintaining a live colony, homozygous mice may be bred together.
When using the Cp- mouse strain in a publication, please cite the originating article(s) and include JAX stock #003582 in your Materials and Methods section.