B6.129-Dag1^tm1Kcam/J

Stock number: 006836
Product name: Dag1 KO
Strain synonyms: TD354 BC6
Research areas: Developmental Biology Research, Neurobiology Research

Description

Homozygous embryos for this dystroglycan 1 knock-out mutant show gross abnormalities, particularly defective Reichert's membrane by 6.5 days.

Detailed description

Heterozygotes are viable and fertile, but homozygous embryos exhibit gross developmental abnormalities beginning around 6.5 days of gestation. This lethality is attributed to a disruption of Reichert’s membrane, an extra-embryonic basement membrane. Laminin and collagen IV are specifically disrupted. No detectable transcript or protein is produced from this allele in homozygous embryos. Northern blot analysis of skeletal muscle RNA shows that transcript levels in heterozygotes are only 10-20% lower than those in wild-type mice. This mutant mouse strain represents a model that may be useful in studies of muscular dystrophies linked to dystrophin-glycoprotein complexes, and developmental processes.

Development

A targeting vector containing a neomycin resistance gene was use to replace a 5' portion of exon 2, including the splice acceptor site and a large part of the coding sequence. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1-Kitl⁺-derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts. Chimeric animals were crossed with C57BL/6. The strain was been backcrossed to C57BL/6 six times by the donating laboratory.

Allele

Symbol: Dag1^tm1Kcam
Name: targeted mutation 1, Kevin P Campbell
MGI accession ID: MGI:1934902
Generation method: Targeted
Attributes: Null/Knockout
Synonyms: Dag1^neo2; DG-
Marker symbol: Dag1
Marker name: dystroglycan 1
Marker synonyms: 156DAG; A3a; AGRNR; alpha-dystroglycan; beta-dystroglycan; D9Wsu13e; DAG; DG; dystrophin associated glycoprotein 1; LGMDR16; MDDGA9; MDDGC7; MDDGC9
Chromosome: 9
Strain of origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺
Molecular note: A neomycin resistance gene replaced part of exon 2, including the splice acceptor site and a large part of the coding sequence. No detectable transcript was produced from this allele in homozygous embryos (data not shown) and immunohistochemistry experiments on homozygous embryos confirmed that no detectable encoded protein was present.