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BCH 380 Seminar: Biochemical Bases of Neurological Disorders, Spring 2010


Instructor
:

Adam C. Hall: x3467, Ford Hall 202A,
Office Hrs: Monday 11-12 Friday 3-4PM or by appointment.

Meetings:
Tuesdays at 1:00-2:50 p.m. in Sabin-Reed 342

 


 

Overview

Following the decade of the brain there has been a surge in understanding of the biochemical and molecular bases of neurological disorders. This seminar will explore the how protein misfolding relates to a number of neuronal diseases including spongiform encephalopathies (e.g. ‘mad cow’), Lou Gehrig's, Alzheimer’s and Parkinson’s.

Pre-requisite: Bio 230, N
3 credits.


Schedule

 

DATES Lectures, Discussions, Presentations and Readings
1/26 Introduction
   
2/2

Background

1) Cellular Neuroscience

2) Protein Folding

3) Overview and General Concepts of Disease

   
2/9 Introduction to Protein Misfolding
   
2/16 Introduction to Transmissible Spongiform Encephalopathy - Student Presentations
   
2/23 Transmissible Spongiform Encephalopathy- Student Presentations
   
3/2 Transmissible Spongiform Encephalopathy- Student Presentations
   
3/9 Introduction to Amyotrophic Lateral Sclerosis-
Student Presentations
   
  Spring Break
   
3/23 Amyotrophic Lateral Sclerosis - Student Presentations
   
3/30 Introduction to Alzheimer’s Disease -Student Presentations
   
4/6 Guest Lecturer – Stephanie Soscia
   
4/13 Introduction to Parkinson’s Disease or Huntington’s-
Student Presentations
   
4/20 Parkinson’s Disease or Huntington’s - Student Presentations
   
4/27 Wrap Up - Student Term Paper Presentations
   

ASSIGNED READINGS

2/2 Background:

1) Cellular Neuroscience: Chapt. 3 and 4, Cellular components of the nervous system and Subcellular organization of the nervous system in Squire et al., Fundamental Neuroscience, 2nd Ed., Elsevier, Academic Press, 2003.

2) Protein Folding: Chapt. 3 Protein Structure and Function, in Berg J. M., Tymoczko, J. L. and L. Stryer, Biochemistry 5th ed., W. H. Freeman and Co., 2002.

3) General Concepts of Disease: Chapt.1 and 2, Cellular Pathology I: Cell Injury and Cell Death and Cellular Pathology II: Adaptations, Intracellular Accumulations and Cell Aging in Cotran R. S., Kumar V and T. Collins Robbins Pathologic, Basis of Disease, 6th ed., W. B. Saunders, Co., 1999.

2/9 Introduction to Protein Misfolding

Soto C., Unfolding the role of protein misfolding in neurodegenerative diseases,Nature Reviews Neuroscience, 4, 49-60 2003. Available SC online.

Ross C.A. and Poirer M.A. Protein aggregation and neurodegenerative disease. Nature Medicine, 10 Suppl., S10-S17, July 2004. Available SC online.

Malgoroli A., Vallar L., Zimarino V. Protein homeostasis in neurons and its pathological alterations. Curr Opin Neurobiol. Jun;16(3):270-4. 2006. Available SC online.

Christopher M. Dobson, Protein misfolding, evolution and disease, Trends in Biochemical Sciences. Volume 24, Issue 9 , 1 September 1999, Pages 329-332

2/16 Introduction to Prions and Spongiform Encephalopathy

Chapt. 224, Prion Diseases by Pruisner in Scriver et al.

Aguzzi A, Polymenidou M., Mammalian prion biology: one century of evolving concepts. Cell 116: 313-327, 2004

Miller G Could they all be Prion Diseases? Science 326: 1337-1339 2009.

2/23 Transmissible Spongiform Encephalopathy

Legname G, Baskakov IV, Nguyen HO, Riesner D, Cohen FE, DeArmond SJ, Prusiner SB, Synthetic mammalian prions. Science 305: 673-676, 2004

Solforosi L, Criado JR, McGavern DB, Wirz S, Sanchez-Alavez M, Sugama S, DeGiorgio LA, Volpe BT, Wiseman E, Abalos G, Masliah E, Gilden D, Oldstone MB, Conti B, Williamson RA., Cross-linking cellular prion protein triggers neuronal apoptosis in vivo. Science 303: 1514-1516, 2004

3/9 Introduction to Amyotrophic Lateral Sclerosis

Cleveland DW and Rothstein JD. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS, Nature Reviews Neuroscience, 2, 806-819, Nov 2001.

Pasinelli P and Brown R H. Molecular Biology of ALS: insights from genetics Nature Reviews Neuroscience, 7, 710-723, Sep 2006.

3/16 Introduction to Amyotrophic Lateral Sclerosis

Readings TBA (based on Dr. Daryl Bosco’s colloquium)

3/23 Amyotrophic Lateral Sclerosis


Ghadge GD, Lee JP, Bindokas VP, Jordan J, Ma L, Miller RJ, Roos RP. Mutant superoxide dismutase-1-linked familial amyotrophic lateral sclerosis: molecular mechanisms of neuronal death and protection. J. Neuroscience, 17: 8756-8766, 1997.


Valentine JS, Hart PJ. Misfolded CuZnSOD and amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. 100: 3617-3622, 2003

3/30 Introduction to Alzheimer’s Disease

Chapter 9, Molecular mechanisms in neurodegenerative disease p202-211 in Molecular Neuroscience, Revest P. and Longstaff A., Bios Scientific 1998.

Masters CL, Cappai R, Barnham KJ, Villemagne VL. Molecular mechanisms for Alzheimer’s disease: implications for neuroimaging and therapeutics J. Neurochem 2006 Jun 97 (6): 1700-25.

Hardy J, Selkoe DJ. The amyloid hypothesis of Alzheimer’s disease: progress and problems on the road to therapeutics 2002 Jul 19: 297 (5580): 353-356.

4/6 Guest Lecturer , Stephanie Soscia
(Readings to be announced)

4/13 Introduction to Parkinson’s Disease

Christopher A. Ross and Cecile M. Pickart, The ubiquitin–proteasome pathway in Parkinson’s disease and other neurodegenerative diseases. Trends in Cell Biology Vol.14 No.12 December 2004.

Maries E., Dass B, Collier TJ, Kordower JH, and Steece-Collier K, The role of œ-synuclein in Parkinson’s disease: insights from animal models. Nature Reviews Neuroscience, 4, 727-738, 2003.

Imai Y and Takahashi R, How do Parkin mutations result in neurodegeneration? Curr. Opin. Neurobio. 14, 384-389, 2004.

4/20 Parkinson’s disease (cont)

GRADE STRUCTURE
The final grade in this course will be computed based on the following distribution:
25 % Class Participation - attendance, discussion, participation, questions, preparedness

25 % Research Article Presentations on topics other than the subject of the term paper

15 % Annotated Bibliography on the specific protein misfolding disease for your term paper

25 % Term Paper

10 % Term Paper Presentation

DEADLINES
Topics for the term paper will be chosen by: 3/23/10
Annotated Bibliography due: 3/30/10 in class
Optional Draft due: 4/09/10 by 4.00PM.
Completed paper is due on: 4/20/10 in class

ARTICLE PRESENTATIONS
Presentations should be about 15 minutes in length and consist of an explanation of the primary literature article you have selected. Emphasize the biochemistry (particularly regarding protein misfolding), cell biology and neuroscience presented in the article. Give a brief background and explain the methodology as needed to interpret the experiments, then discuss how the knowledge gained elucidates some aspect of the disease. This is a Powerpoint presentation with handouts as needed to clarify the material. Please email me the Powerpoint slides 24 hrs before the presentation date, (i.e. by 4 p.m the Monday before).

ANNOTATED BIBLIOGRAPHY
An annotated bibliography is a combination of the complete bibliographic citation along with several sentences (5-10) of concise summary of that article IN YOUR OWN WORDS. This usually includes the most important results and the relevance of the study to the field. The articles you choose will be ones that you are using or have considered for use in your term paper. You should have 5 - 10 references of primary literature in your annotated bibliography. Any reviews cited do not require annotation.

 

TERM PAPER
Format: The paper should be typed on white paper, double-spaced, 1 inch margins, 12 pitch Times-Roman, paginated and up to 12 pages in length (exclusive of figures and bibliography). Genus and species names are to be italicized as are Latin abbreviations and words, such as et al., in vivo, in vitro.


I’d be happy to read a draft of your paper. Comments will be made on the depth of the coverage, the logical flow and the content (but not checked for grammar and spelling)


Abstract: An abstract of no more than 200 words should be included as the first page.
Coverage: The discussion of the disease should include:

1. A brief definition of the disease and clinical symptoms

2. A brief historical perspective and discussion of the development of our changes in understanding of the disease.

3. A presentation of the BIOCHEMICAL, CELLULAR, AND METABOLIC pathways involved in the disease, particularly as it relates to PROTEIN MISFOLDING IN THE NERVOUS SYSTEM.


Documentation:
The use of proper and complete referencing and documentation is important. The references cited should be given in the format given below. Be sure to include all authors.

Author, A. B. (complete listing of all authors), Year, Title of the article. Abbreviated Journal Title, Vol.: Complete Pagination.


Within the body of the paper, please refer to the references by author and year within parentheses. For example, this syllabus was prepared at Smith College (Hall, 2006). Hall et al., (2006) have established that menthol can act as an anesthetic in vivo….

TERM PAPER PRESENTATION
This short presentation (maximum of 10 minutes) will consist of a summary which describes the cell biology and biochemistry of the cells/tissue involved in the disease. Then a description of the pathology and abnormalities at the morphological, cellular and biochemical levels. Emphasize the biochemical bases of the disease again with a focus on protein misfolding. No more than five Powerpoint slides may be used. Please email me the Powerpoint slides 24 hours before the presentation (i.e. on Monday April 26 by 4 p.m).


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