Three Novel Immune-deficient Mouse Models of Muscular Dystrophy

This study provides three novel severely immune-deficient mouse muscular dystrophy models that will be useful in the development of successful gene and cell therapies. We also characterize the models to provide valuable baseline phenotypic information about them. Comparing the results between strains may elucidate further the phenotypic differences between these three forms of muscular dystrophy, although we note that since the strains are not fully inbred, differences in modifier genes may be present between them that could affect phenotype. Materials and Methods Ethics statement The Stanford Administrative Panel on Laboratory Animal Care approved all procedures performed on animals in protocol number 15766, assurance number A3213-01. The Stanford Comparative Medicine program is accredited by the Assessment of Laboratory Animal Care International. Mouse strains NOD (NOD/ShiLtJ, 001976), NRG (NOD.Cj-Rag1tmMomIl2rγtm1Wjl/Szj, 007799) 28, mdx4Cv (B6Ros.Cg-Dmdmdx-4Cv/J, 002378) 30, and mdx/scid (B10ScSn.Cg-PrkdcscidDmdmdx/J) mice were purchased from the Jackson Laboratory (Bar Harbor, ME). Bl/AJ mice (B6.A-Dysfprmd/GeneJ, 012767) 6 were provided by the Jackson Laboratory from a stock maintained by the Jain Foundation. SGCA-null mice 3 were a kind gift from Kevin Campbell. PCR analysis of genotypes Genomic DNA was extracted from mouse ear punch using Wizard Genomic DNA Purification Kit according to manufacture’s instructions (Promega, Cat. A1120). IL2rÎ...
Source: PLOS Currents Muscular Dystrophy - Category: Neurology Authors: Source Type: research