Research articleDoes utrophin expression in muscles of mdx mice during postnatal development functionally compensate for dystrophin deficiency?
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Overview of preclinical research
2023, Translational Sports MedicineImplications of increased S100β and Tau5 proteins in dystrophic nerves of two mdx mouse models for Duchenne muscular dystrophy
2020, Molecular and Cellular NeuroscienceCitation Excerpt :In mature innervated normal myofibres utrophin is restricted to the sarcolemma beneath the NMJ, and is also found at myotendinous junctions, in peripheral nerves and the vasculature (Vater et al., 1998). In developing immature myotubes and myofibres, utrophin is present around all the sarcolemma but becomes restricted to the NMJ as myofibres mature: this occurs by 3 weeks after birth in both normal and mdx mice (Pons et al., 1994; Khurana et al., 1991; Law et al., 1994). However after 3 weeks in mdx mice, in response to the acute onset of myonecrosis the utrophin protein is markedly increased in new myotubes and regenerating myofibres (Pons et al., 1994; Khurana et al., 1991; Law et al., 1994) and can remain elevated around the sarcolemma of regenerated myofibres.
“Known Unknowns”: Current Questions in Muscle Satellite Cell Biology
2018, Current Topics in Developmental BiologyCitation Excerpt :Mice, unlike humans, are also commonly analyzed as inbred strains, which can possess different intrinsic characteristics that would be expected to modify experimental results (Grounds & McGeachie, 1989; Irintchev & Wernig, 1987). Mice also have higher expression of the utrophin protein, which may compensate for a defective dystrophin (Pons, Robert, Marini, & Leger, 1994), and their satellite cells (like all mouse cells) have telomeres that are ~ 50-fold longer than a human's (Prowse & Greider, 1995). Evidence for each of these as a disease-modifying factor in mouse comes from mutant strains carrying multiple mutations: mdx/utrophin knockout mice (Grady et al., 1997) or mdx/telomerase knockout mice (Sacco et al., 2010) have a much more severe phenotype, approaching that of the human disease state.
α7β1 integrin does not alleviate disease in a mouse model of limb girdle muscular dystrophy type 2F
2007, American Journal of PathologyUtrophin lacks the rod domain actin binding activity of dystrophin
1999, Journal of Biological ChemistryDystrophin is required for organizing large acetylcholine receptor aggregates
1999, Brain Research