【学习ppt课件】神经系统退行性疾病基础.ppt

Neurodegenerative Diseases;What Is Neurodegenerative diseases;Neurodegenerative Diseases;大脑皮层变性：包括Alzheimer病、Pick病、Creutzfeldt?Jakob病(海绵状变性)等。 锥体外系统变性：包括Huntington病、Hallervorden?Spatz病、Wilson病(肝豆状核变性)、Seitelberger病(神经轴索型营养不良)、进行性肌阵挛型癫痫。病损在中脑与纹状体者有Parkinson(帕金森)病、纹状体黑质变性、进行性核上型麻痹(PSP)等。 脑干小脑变性：包括各种小脑型共济失调、脊髓小脑变性、橄榄?桥脑?小脑变性(OPCA)、Machado?Joseph病等。 脊髓变性：包括进行性痉挛性截瘫、进行性后索变性、后侧索联合变性、Friedreich共济失调等。 运动系统变性：包括各型运动神经元病，如肌萎缩侧索硬化(ALS)、进行性脊髓性肌萎缩(SMA)、进行性球麻痹等。 自主神经系统变性：包括Riley?Day症候群(全自主神经功能不全)、Shy?Drager症候群等。 多系统变性(MSA)：包括上述1、2、3、6等的混合类型。;Parkinson disease;崔德华 DH Chui;What Is Alzheimer Disease ? ;Alzheimer Disease;Growth in U.S. Population Aged 65+, 75+, and 85+;Genes Associated with Alzheimer Disease;Classification of Senile Dementia;The AD diagnosis;AD clinical symptom; ;;崔德华 DH Chui;;HowDiscrimination  Between Earlier Period AD and Age-Associated Memory Impairment in Aging; 1986年美国国立精神保健研究所提出: AAMI Age-Associated Memory Impairment @ 随年龄增加出现非病理性的记忆力下降 @ 健忘是老年人脑功能衰弱的表现. @ 痴呆则是病理性的脑器质性智能衰退。 ;如何区分老年健忘与早期AD;;;;Schematic representation of regional and laminar NFT formation and neuronal loss in normal aging and AD;What isPaired Helical Filaments Tau？ - PHF Tau -;a) The cytoskeleton b) components of the cytoskeleton;Sulfo-glycosaminoglycan content affects PHF-tau solubility and allows the identification of different types of PHFs;崔德华 DH Chui;崔德华 DH Chui;APP : Amyloid precursor protein;What is Presenilin, APP and Ab ?  ;Molecular features of presenilin and APP;Molecular features of APP and Ab peptides;presenilin (Psn）;Aggregation of ?-amyloid is a multi step process;Courtesy: Prof. C. Glabe, UC Irvine;Proposed actions of heat shock protein 70 and heat shock protein 40 chaperones on amyloid assembly;Direct and indirect effects of molecular chaperones on disease protein toxicity; Protein misfolding diseases associated with molecular chaperones ; PresenilinAb cascades in AD ;;;;;;;;Formation of TAU inclusions in knock-in mice with familial Alzhe