Dmd-Q995X

Nomenclature

C57BL/6Smoc-Dmdem1(Q995X)Smoc

Cat. NO.

NM-KI-18026

Strain State

Repository Live

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Gene Summary

Gene Symbol
Dmd

Model Description

These mice carry a p.Q995X mutation in Dmd gene.

Disease Connection

Duchenne muscular dystrophy

Validation Data

dmd-1.png

Fig1 Mouse dystrophin expression is abolished due to the p.Q995X mutation.

mdx.png

Fig2 Histopathology of WT and MDX mice(male, 6-month-old) muscle showing less uniform muscle fibers with inflammation and clustered nuclei.

mdx-2.png

Fig3 Limb grip strength tests in MDX mice.

The assessment of limb strength in experimental mice were performed by means of grip strength meter (BIO-G53, Bioseb, France). The mice were allowed to grab the pull bar and are then gently pulled backwards. The force applied to the bar just before it loses grip was recorded as peak resistance force (expressed in grams). To reduced procedure-related variability, the tests were repeated 4 to 5 times and the average was recorded.


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