Dmd-Q995X

Background : C57BL/6Smoc

Catalog : NM-KI-18026

Strain Name :

C57BL/6Smoc-Dmd^{em1(Q995X)Smoc}

Gene Summary

Gene Name

Synonyms :
dys, mdx, pke, Dp71, Dp427, DXSmh7, DXSmh9
NCBI ID :
13405
MGI ID :
94909
Ensembl ID :
ENSMUSG00000045103
Strain State :
Repository Live

Model Description

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

Validation Data

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

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

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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