PHYSCI 121 Lecture Notes - Lecture 16: Dystrophin, Transgene, Sarcolemma
Document Summary
Utrophin is a dystrophin paralog and is expressed around the extra-synaptic sarcolemma in mdx and. Way to address this is to create mouse that lacks utrophin. Experiment: took mdx mice and crossed them with mice without the utrophin gene. We describe mice deficient for both dystrophin and the dystrophin-related protein utrophin. These mice show many signs typical of dmd in humans: they show severe progressive muscular dystrophy that results in premature death, they have ultrastructural neuromuscular and myotendinous junction abnormalities The data suggest that utrophin and dystrophin have complementing roles in normal functional or developmental pathways in muscle. Dko mice have very severe symptoms of the disease. Normal mice are the same size as the mdx mice. Dko mice are smaller than the mdx mice. Curvature of the spine is different because of the muscle weakness. Dko mice die after a few weeks to a few years of age compared to the mdx mice that live a normal life.
