2022
DOI: 10.1007/s00018-022-04151-2
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Generation of dystrophin short product-specific tag-insertion mouse: distinct Dp71 glycoprotein complexes at inhibitory postsynapse and glia limitans

Abstract: Duchenne muscular dystrophy (DMD), the most severe form of dystrophinopathies, is a fatal X-linked recessive neuromuscular disorder characterized by progressive muscle degeneration and various extents of intellectual disabilities. Physiological and pathological roles of the responsible gene, dystrophin, in the brain remain elusive due to the presence of multiple dystrophin products, mainly full-length dystrophin, Dp427, and the short product, Dp71. In this study, we generated a Dp71-speci c hemagglutinin (HA) … Show more

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Cited by 8 publications
(2 citation statements)
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“…Because the C272Y and E299del mutations are mapped on the β-dystrolgycan binding domain of Dp71 [ 39 , 40 , 41 ], we analyzed the impact of expressing these Dp71 mutants on the subcellular localization of β-DG. The distribution of β-DG was partitioned between the nucleus and the cytoplasm in SH-SY5Y/N1E-115 cells expressing GFP alone or GFP-Dp71; however, a polarized cytoplasmic distribution of β-DG with some aggregates was commonly observed in SH-SY5Y/N1E-115 cells expressing both Dp71 mutants ( Figure 4 ).…”
Section: Resultsmentioning
confidence: 99%
“…Because the C272Y and E299del mutations are mapped on the β-dystrolgycan binding domain of Dp71 [ 39 , 40 , 41 ], we analyzed the impact of expressing these Dp71 mutants on the subcellular localization of β-DG. The distribution of β-DG was partitioned between the nucleus and the cytoplasm in SH-SY5Y/N1E-115 cells expressing GFP alone or GFP-Dp71; however, a polarized cytoplasmic distribution of β-DG with some aggregates was commonly observed in SH-SY5Y/N1E-115 cells expressing both Dp71 mutants ( Figure 4 ).…”
Section: Resultsmentioning
confidence: 99%
“…DP71 localizes to astrocyte endfeet. 34,35 Because endfeet directly contact blood vessels, DP71 can be used as a protein marker for visualization. 36 Anti-DP71 (Figure 3(e)) showed moderate vessel staining, but also had a moderately high background, including staining of cell bodies.…”
Section: Ffpe Stainingmentioning
confidence: 99%