STOCK Cp^tm1Hrs/J

Stock number: 003582
Product name: Cp-
Research areas: Hematological Research, Metabolism Research, Mouse/Human Gene Homologs

Description

These Cp knock-out mice exhibit a progressive accumulation of iron in hepatocytes and reticuloendothelial cells.

Detailed description

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.

Development

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

Symbol: Cp^tm1Hrs
Name: targeted mutation 1, Z Leah Harris
MGI accession ID: MGI:1857981
Generation method: Targeted
Attributes: Null/Knockout
Marker symbol: Cp
Marker name: ceruloplasmin
Chromosome: 3
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.