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Phenotypes Associated with This Genotype
Genotype
MGI:4361735
cx34
Allelic
Composition
Dmdmdx/Dmdmdx
Spp1tm1Blh/Spp1tm1Blh
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * C57BL/10 * C57BL/10ScSn
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation (31 available); any Dmd mutation (156 available)
Spp1tm1Blh mutation (2 available); any Spp1 mutation (43 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Reduced diaphragm and cardiac fibrosis in Dmdmdx/Y Spp1tm1Blh/Spp1tm1Blh and Dmdmdx/Dmdmdx Spp1tm1Blh/Spp1tm1Blh mice compared to Dmdmdx/Dmdmdx mice

immune system
• muscles contain fewer granulocytes than in Dmdmdx homozygotes
• muscles contain fewer neutrophils than in Dmdmdx homozygotes
• muscles contain fewer CD3+/Vbeta8.1/8.2+, and NKT-like cells than in Dmdmdx homozygotes
• muscles contain more CD3+ T cells than in Dmdmdx homozygotes
• muscles contain more CD3+CD4+ regulatory T cells than in Dmdmdx homozygotes

muscle
• mice exhibit improved muscle regeneration and reduced muscle inflammation and fibrosis in the diaphragm and heart compared with Dmdmdx homozygotes

behavior/neurological
• at 4 and 8 weeks, mice exhibit increased strength on a wire and grip test compared with Dmdmdx homozygotes

hematopoietic system
• muscles contain fewer granulocytes than in Dmdmdx homozygotes
• muscles contain fewer neutrophils than in Dmdmdx homozygotes
• muscles contain fewer CD3+/Vbeta8.1/8.2+, and NKT-like cells than in Dmdmdx homozygotes
• muscles contain more CD3+ T cells than in Dmdmdx homozygotes
• muscles contain more CD3+CD4+ regulatory T cells than in Dmdmdx homozygotes


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
03/18/2025
MGI 6.24
The Jackson Laboratory