课件the molecular basis of muscular dystrophy（肌营养不良）

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1、Themolecularbasisofmusculardystrophy(肌营养不良)WenyaHouXueJingYitangWangJiezhongZhangOUTLINEINTRODUCTIONDuchennemusculardystrophy(DMD)DysfelinTherapeuticApproachesandperspectiveaquarterofamillionkidsandadultsarelivingwiththedisease,sochancesareyoumayknowsomeonewhohasit.W

2、hatisMuscularDystrophy?Themusculardystrophies(MD)areagroupofmorethan30geneticdiseasescharacterizedbyprogressiveweaknessanddegenerationoftheskeletalmusclesthatcontrolmovement.SomeformsofMDareseenininfancyorchildhood,whileothersmaynotappearuntilmiddleageorlater.Muscular

3、dystrophiesingeneralareagroupofgenetic,degenerativediseasesprimarilyaffectingvoluntarymuscles.Healthymuscletissue(left).Musculardystrophy(right).HOWTORECOGNIZEIFMUSCLEWEAKNESSISCAUSEDBYMUSCULARDYSTROPHYMostlyaffectsboys(rarelygirls).Oftenbrothersormalerelativeshavesam

4、eproblem.Firstsignsappeararoundages3to5:thechildmayseemawkwardorclumsy,orhebeginstowalk'tiptoe'becausehecannotputhisfeetflat.Runsstrangely.Fallsoften.Problemgetssteadilyworseoverthenextseveralyears.HOWTORECOGNIZEIFMUSCLEWEAKNESSISCAUSEDBYMUSCULARDYSTROPHYMuscleweaknes

5、sfirstaffectsfeet,frontsofthighs,hips,belly,shoulders,andelbows.Later,itaffectshands,face,andneckmuscles.Mostchildrenbecomeunabletowalkbyage10.Maydevelopaseverecurveofthespine.Heartandbreathingmusclesalsogetweak.Childusuallydiesbeforeage20fromheartfailure.Thereareprob

6、ablyninetypesofmusculardystrophy.Duchennemusculardystrophy(DMD)Beckermusculardystrophy(BMD)Emery-Dreifussmusculardystrophy(EDMD)Limb-girdlemusculardystrophy(LGMD)Facioscapulohumeralmusculardystrophy(FSHD)Myotonic(pronounced:my-uh-tah-nick)dystrophy(MMD)Oculopharyngeal

7、MuscularDystrophy(OPMD)DistalMuscularDystrophy(DD)Congenitalmusculardystrophy(CMD)Duchennemusculardystrophy(DMD)Definition-Oneofninetypesofmusculardystrophy,agroupofgenetic,degenerativediseasesprimarilyaffectingvoluntarymuscles.Cause-Anabsenceofdystrophin,aproteinthat

8、helpskeepmusclecellsintact.DMDOnset-Earlychildhood-about2to6years.Symptoms-Generalizedweaknessandmusclewastingfirstaffecting