This spontaneous mutation of Dmd is one several Dmdmdx revertants and exhibits muscle fiber necrosis, fibrosis and centrally nucleated skeletal muscle fibers. This allele has 10-fold fewer revertants than mdx-2Cv and mdx-3Cv and may be useful in studies of muscular dsystrophy.
Dr. Verne M. Chapman (deceased), Roswell Park Memorial Institute
The Dmdmdx-4Cv and Dmdmdx-5Cv strains have 10 times fewer revertants than the Dmdmdx and Dmdmdx-2Cv strains as viewed in quadricep cross-sections. This is not attributable to genetic background or viral infections. These reversion rate differences may be attributable to differences in the location of the point mutation. The large number of revertants in Dmdmdx mutants has complicated the analysis of gene or cell therapies. These mutants are more useful for this purpose. All these strains are also hemizygous for Hprta and Pgk1a (both are on the X chromosome).
This strain was created in the laboratory of Verne M. Chapman. A C57BL/6Ros female was crossed to a male of strain C3Ha.X25, a double congenic strain carrying Pgk1a (from a wild Mus musculus musculus mouse trapped in Denmark) and Hprta (from Mus castaneus) on a C3H/HeHa background. F1 or F2 male progeny of this cross were treated with n-ethylnitrosourea (ENU) and crossed to C57BL/10Sn-Dmdmdx/+ females. Female offspring of these crosses that exhibited consistently elevated plasma creatine kinase levels and that carried the X-chromosome markers of their mutagenized male progenitors were bred to C57BL/10Sn-Dmdmdx/Y males. Transmission to male progeny of the elevated plasma CK phenotype and failure of the suspected new mutations at the Dmd locus to complement the classical mdx mutation identified four new mutations of Dmd, called Dmdmdx-2-5Cv . Each of these new mutations was subsequently backcrossed onto C57BL/6Ros.
|Allele Name||X linked muscular dystrophy 4, Verne Chapman|
|Allele Type||Chemically induced (ENU)|
|Allele Synonym(s)||mdx4cv; mdxCv4; mdx4cv; mdx4cv|
|Gene Symbol and Name||Dmd, dystrophin, muscular dystrophy|
|Strain of Origin||C3Ha.Cg-Hprta Pgk1a|
|Molecular Note||A C to T transition in exon 53 at position 7916 creates a premature stop codon.|
|Mutations Made By|| |
Dr. Verne Chapman (deceased), Roswell Park Memorial Institute
Shin JH, Hakim CH, Zhang K, Duan D. 2011. Genotyping mdx, mdx3cv, and mdx4cv mice by primer competition polymerase chain reaction. Muscle Nerve 43(2):283-6. [PubMed: 21254096]
This strain is maintained by mating homozygous females to hemizygous males (X-linked).
When using the X linked muscular dystrophy 4 mouse strain in a publication, please cite the originating article(s) and include JAX stock #002378 in your Materials and Methods section.
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