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Univ. of Pennsylvania Penn's Neurobiology of Disease Curriculum

WWW NBD
FELLOWSHIPS available to train in Translational Research in NBD
NEURODEGENERATIVE DISEASES II - INSC 600-001
Triplet repeat disorders, inherited neuropathies, muscular dystrophies, Friedreich ataxia, lysosomal storage diseases, mitochondrial disorders
2009 schedule is M/W 2:00-3:30; Beginning Mar 9 (Mon) through Apr 22 (Wed)

Module Overview

Neurodegenerative diseases represent a major area of advance over the past 10 years. During this time, a variety of technique and pathophysiological mechanisms have become common to different degenerative disorders prompting the need for studying them in an integrative, detailed fashion. This module will use detailed critical literature assessment guided by expert faculty members along with faculty seminar like presentations to present recurring themes (protein aggregation, reactive oxygen species production, genetic risk modifiers) as well as differing mechanisms among diseases. This module will examine known genetic disorders, including triple repeat disorders (HD, spinocerebellar ataxias), inherited neuropathies, muscular dystrophies, Friedreich ataxia, lysosomal storage diseases and mitochondrial disorders. These topics will provide a framework for investigating more common sporadic degenerative disorders - Alzheimer's disease, Parkinson disease, amyotrophic lateral sclerosis - that are covered in Neurodegenerative Diseases I.

Module Goals

Viewing the Lectures from 2009

From a PC: Just click on the link
From a Mac: download - just once - a copy of silverlight.

Lectures - Spring 2009

1. Introduction to genetic neurodegenerative diseases; Introduction to repeat expansion diseases (REDs); RED classification; Dr. Lynch SCA patient Mon Mar 9 Video/Slides
2. Triplet repeat diseases: Disease specific mechanisms in triplet repeat disease ROS productionx Dr. Lynch Wed Mar 11 Video/Slides
3. Mechanistic aspects of type 1 and 2 REDs. Fragile X Dr. Bonini Mon Mar 16 Video/Slides
4. Mechanistic aspects of type 3 REDs 5. Friedreich ataxia Dr. Lynch Friedreich ataxia patient Wed Mar 18 Video/Slides
5. Friedreich ataxia-basic and clinical science Dr. Wilson Mon Mar 23 Video/Slides
6. Neurobiology of the Tuberous Sclerosis Complex Dr. Crino; Patient or discussion Wed Mar 25 Video/Slides
7. Motor neuron diseases Dr. Kalb; Patient or discussion Mon Mar 30 Video/Slides
8. Peripheral Neuropathies Dr. Scherer; Patient with neuropathy Wed Apr 1 Video/Slides
9. Hereditary Spastic Paraparasis Dr. Scherer Mon Apr 6 Video/Slides
10. Neuromuscular junction disorders Dr. Bonnemann Wed Apr 8 Video/Slides not available
11. Muscle disease Dr. Bonnemann Mon Apr 13 Video/Slides not available
12. Neuromuscular Junction Disorders Dr. Jon Lindstrom Wed Apr 15 Video/Slides
13. Gene therapy and lysosomal disorders Dr. Watson Mon Apr 20 Video/Slides

Viewing the Lectures from 2007

For helpful hints on viewing the videotaped lectures go HERE.
You will need RealPlayer to view the 2007 videos. Make sure to click "Free Download."

Lectures from Neurodegenerative Diseases II - Spring 2007

Lecture Title Lecturer Date Real
video
Slides
only
1. Introduction to genetic neurodegenerative diseases Dr. Lynch Tues Feb 27 link to video link to slides
2. Introduction to repeat expansion diseases (REDs); RED classification; mechanistic aspects of type1 REDs. Dr. Taylor: Kennedy's disease patient visit Thurs Mar 1 link to video link to slides
3. Friedreich ataxia Dr. Lynch: Friedreich ataxia patient visit Tues Mar 6 link to video link to slides
4. Triplet repeat diseases: Disease specific mechanisms in triplet repeat disease ROS production Dr. Lynch Thurs Mar 8 link to video link to slides
5. Friedreich ataxia-basic and clinical science Dr. Wilson Tues Mar 13 link to video link to slides
6. Mechanistic aspects of type 2 and 3 REDs Dr. Taylor Thurs Mar 15 class to be rescheduled  
7. Congenital Myopathies: Nemaline Myopathy; Myotubular Myopathy; Central Core Myopathy Dr. Bonnemann Tues Mar 20 no video or slides  
8. Neuromuscular junction disorders Dr. Lindstrom Tues Mar 22 cancelled  
9. Inherited Neuropathies Dr. Scherer Tues Mar 27 link to video link to slides
10. Duchenne Muscular dystrophies Dr. Bonnemann Thurs Mar 29 no video or slides  
11. Mechanistic aspects of type 2 and 3 REDs Dr. Taylor Tues Apr 3 link to video link to slides
12. Mitochondrial disease Dr. Lynch Thurs Apr 5 link to video link to slides
13. Gene therapy in the Central Nervous System (and Lysosomal disorders) Dr. Wolfe Tues Apr 10 link to video link to slides
14. Hereditary spastic paraparesis and new directions in the Neurobiology of the Tuberous Sclerosis Complex Dr. Crino Thurs Apr 12 link to video link to slides
15. Gene therapy for neurodegenerative disease Dr. Wolfe Tues Apr 17 link to video link to slides