词汇 | dystrophin |
释义 | BETA Examples of dystrophindystrophin isn’t in the Cambridge Dictionary yet. You can help! Intracellularly, -dystroglycan is linked to the actin cytoskeleton by its interaction with dystrophin or utrophin. Dystrophin expression in the human retina is required for normal function as defined by electroretinography. Dystrophin localization at presynapse in rat retina revealed by immunoelectron microscopy. New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin. Without dystrophin to strengthen them, the muscle cells become weaker and eventually waste away. From the Hansard archive Example from the Hansard archive. Contains Parliamentary information licensed under the Open Parliament Licence v3.0 Loss of this complex as a result of mutations in dystrophin or one of the associated proteins leads to muscular dystrophy (fig001dwl). These patients might not be tolerant of dystrophin gene transfer. The increased capacity of these vectors, which contain no viral coding sequences, makes them suitable for gene transfer of full-length dystrophin. This raises the possibility that the expression of dystrophin following gene transfer might trigger a destructive immune response against this 'neoantigen'. Thus, dystrophin and the associated proteins are thought to have both a structural and a signalling role in the muscle fibre. The current evidence for immune responses to newly expressed dystrophin and the vector itself is discussed here in more detail. Although some patients might show tolerance to restoration of dystrophin expression, it is probable that most will not. What are the functional consequences of loss of dystrophin or syntrophin on seizure susceptibility? The full coding sequence for dystrophin is 11.5 kb, which was too large for most of the viral vectors that were available in the early 1990s. Dystrophin forms a link between the actin cytoskeleton and the extracellular matrix via the dystroglycans. |
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