文档介绍:-fiberdegenerationandresultingmuscle变性,变质,,calledDuchenne杜兴肌肉萎缩症musculardystrophyafterthepersonwhodescribeditin1861,mon,debilitatingdisorderthatstrikes逐渐衰弱的病症,不舒about1in3,“muscular相关联的dystrophy,”,顿悟3TheMolecularBasisofMuscularDystrophybetweenthecytoskeleton,plasmamembrane,(DMD).,andithasthedistinctionofbeingthe抗肌肉萎缩蛋白largestgenesofaridentifiedinthemammalian 已鉴定的哺乳动物的 ,alengththatrequires16hourstobetranscribedintoasingle转录mRNA!;()(page149),dystrophinmoleculesarerod-shapeddimers杆状二聚物thatliejustbeneaththeplasmamembrane(Figure1).6TheMolecularBasisofMuscularDystrophyOntheircytoplasmicside,dystrophinmoleculesareattachedtoactinfilaments,andontheir肌动蛋白纤丝束membraneside,theyareattachedtoaclusterofdystophin-associatedproteins(DAPs),inturn,,会收缩的,,