Most Cby1 (chibby homolog 1 (Drosophila)) targeted mutant homozygotes die before or around weaning age. The mice develop rhinitis and sinusitis and are unable to clear Pseudomonas aeruginosa bacteria from the nasal cavity. There is a complete absence of mucociliary transport caused by a marked lack of motile cilia in the nasal epithelium. This strain may be a useful model of primary ciliary dyskinesia.
Randall Moon, University of Washington, HHMI
Homozygous targeted mice are runted and demonstrate a failure to thrive. Most die before or around weaning age. Mice surviving after postnatal day 25 (P25) usually recover and apparently have normal life spans. All of the mice develop rhinitis and sinusitis and are unable to clear Pseudomonas aeruginosa bacteria from the nasal cavity. There is a complete absence of mucociliary transport caused by a marked lack of motile cilia in the nasal epithelium. Anemia and ductal dilation in the pancreas and kidneys is also observed. Homozygous males have compromised fertility. This strain may be useful as a model of primary ciliary dyskinesia.
The four coding exons were replaced by a neomycin resistance cassette using the (129X1/SvJ x 129S1/Sv)F1- Kitl+-derived R1 embryonic stem (ES) cell line. The strain was backcrossed to C57BL/6 for 5 generations by the donating laboratory.
|Allele Name||targeted mutation 1, Ken-Ichi Takemaru|
|Allele Type||Targeted (Null/Knockout)|
|Gene Symbol and Name||Cby1, chibby family member 1, beta catenin antagonist|
|Strain of Origin||(129X1/SvJ x 129S1/Sv)F1-Kitl+|
|Molecular Note||The coding region was replaced with a neo cassette.|
|Mutations Made By|| |
Ken-Ichi Takemaru, State University of NY at Stony Brook
When maintained as a live colony, heterozygotes may be bred. Homozygotes have a high rate of postnatal mortality. Surviving homozygous males have compromised fertility and homozygous females produce pups, but make poor mothers.
When using the B6.129-Cby1tm1Ktkm/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #009644 in your Materials and Methods section.