B6.129-Jup^tm1Kem/J

Stock number: 003360
Product name: plako-
Research areas: Cardiovascular Research, Cell Biology Research, Dermatology Research, Developmental Biology Research, Internal/Organ Research

Description

These Jup knock-out mice exhibit embryonic or early death with skin blistering and subcorneal acantholysis.

Detailed description

Plakoglobin Jup null-mutant embryos die from embryonic day 10.5 onward due to severe heart defects. Some mutant embryos develop further, especially on a C57BL/6 background. These mice die around birth, presumably due to cardiac dysfunction, and with skin blistering and subcorneal acantholysis. The skin phenotype in plakoglobin-deficient mice is reminiscent of the human blistering disease, epidermolytic hyperkeratosis.

Development

A targeting vector containing the neomycin resistance cassette was used to replace exons 3 and 4. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1 derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts. The resulting chimeric animals were bred to crossed to C57BL/6J and offspring were backcrossed to C57BL/6J for at least 7 generations.

Allele

Symbol: Jup^tm1Kem
Name: targeted mutation 1, Rolf Kemler
MGI accession ID: MGI:1857539
Generation method: Targeted
Attributes: Null/Knockout
Marker symbol: Jup
Marker name: junction plakoglobin
Chromosome: 11
Strain of origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺
Molecular note: A neomycin resistance cassette replaced exons 3 and 4 of the gene.