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Neurodegenerative Disorders
 
January 28 - February 1
Ana C. Rego, Cláudia Pereira, Luís P. Almeida, Paula Agostinho, Paula Moreira, Sandra M. Cardoso
Invited Lecturers:


David Borchelt -Department of Neuroscience, McKnight Brain Institute, Univ. of Florida, Gainesville, FL, USA (borchelt@mbi.ufl.edu)


Sebastian Kügler -Department of Neurology, Faculty of Medicine, S2-Laboratory, University of Göttingen, Waldweg 33, 37073 Göttingen, Germany (sebastian.kuegler@med.uni-goettingen.de)


Cristina Januário -Serviço de Neurologia, Hospitais da Universidade de Coimbra (cristinajanuario@gmail.com)


Isabel Santana -Serviço de Neurologia, Hospitais da Universidade de Coimbra (isabelsantana@huc.min-saude.pt)
Luís Negrão -Serviço de Neurologia, Hospitais da Universidade de Coimbra (luis.negrao@netvisao.pt)


Paula Coutinho -UnIGENe, IBMC, Departamento de Neurologia, Hospital de São Sebastião, Universidade do Porto, 4520-211 Santa Maria da Feira (pcoutinho@hospitalfeira.min-saude.pt)


Sandra Macedo Ribeiro -Instituto de Biologia Molecular e Celular, Porto (sribeiro@ibmc.up.pt)


Tiago Outeiro -Harvard Medical School, Boston, USA and Instituto de Medicina Molecular, Lisboa, Portugal (touteiro@gmail.com)


Course Schedule:

Monday (Jan 28)
9:30-10:00- Overview of general aspects of neurodegenerative disordersDavid Borchelt
10:00-10:30- Alzheimer’s disease: diagnosis and clinical presentationIsabel Santana
10:30-11:00- Break
11:00-11:30- Parkinson´s disease: pathophysiology of clinical signs
Cristina Januário
11:30-12:00- Clinical aspects of amyotrophic lateral sclerosisLuís Negrão
12:00-14:00- Lunch and social event
14:00-14:30- Clinical features of Huntington’s diseaseCristina Januário
14:30-15:00- Clinical aspects of Machado-Joseph diseasePaula Coutinho
15:00-15:15- Presentation of student’s assignments

Tuesday (Jan 29)
9:30-10:30- Animal models of Alzheimer’s disease – evolution of AD pathologyDavid Borchelt
10:30-11:00- Break
11:00-11:30- Mitochondrial dysfunction in Alzheimer’s diseasePaula Moreira
11:30-12:00- Endoplasmic reticulum stress during Abeta- induced cell deathCláudia Pereira
12:00-12:30- Glial activation triggered by Abeta: role in AD associated neuronal dysfunctionPaula Agostinho
12:30-14:00- Lunch
14:00-15:00- Modelling motor neurodegeneration in transgenic and knockout mouse modelsDavid Borchelt
15:00-15:30- Break
15:30-16:30- Targeting of viral gene transfer vectors: the AAV toolkitSebastian Kuegler

Wednesday (Jan 30)Protein misfolding in biology and diseaseTiago Outeiro
10:00-11:00-
11:00-11:30- Break
11:30-12:00- The role of mitochondria in Parkinson’s diseaseSandra Morais Cardoso
12:00-14:00- Lunch
14:00-15:00- Molecular strategies to study Parkinson´s disease: from yeast to miceTiago Outeiro
15:00-15:30- Break
15:30-16:30- Gene transfer by viral vectors for generation of animal models of CNS disordersSebastian Kuegler

Thursday (Jan 31)Modeling HD in transgenic mice – role of huntingtin proteolytic processing in pathophysiologyDavid Borchelt
9:30-10:30-
10:30-11:00- Break
11:00-11:45- Mitochondrial dysfunction and oxidative stress in Huntington´s diseaseAna Cristina Rego
11:45-12:45- Gene therapy of nonogenic disorders: molybdenum cofactor deficiency and neuronal ceroid lipofuscinoses
Sebastian Kuegler
12:45-14:30- Lunch
14:30-15:15- Modelling and silencing Machado-Joseph disease by lentiviral gene transferLuís Pereira de Almeida
15:15-15:30- Break
15:30-16:30- Exploring the role of the protein context in Machado-Joseph´s diseaseSandra Ribeiro
16:30-17:30- Paper presentation by students


Paper 1
* Santacruz K, Lewis J, Spires T, Paulson J, Kotilinek L, Ingelsson M, Guimaraes A, DeTure M, Ramsden M, McGowan E, Forster C, Yue M, Orne J, Janus C, Mariash A, Kuskowski M, Hyman B, Hutton M, Ashe KH. (2005)Tau suppression in a neurodegenerative mouse model improves memory function. Science. 309:476-81.

* [Supplementar information]
Students: Ana Sofia Cabral and Gil Cunha


Paper 2
* Gorbatyuk OS, Li S, Sullivan LF, Chen W, Kondrikova G, Manfredsson FP, Mandel RJ, Muzyczka N. (2008) The phosphorylation state of Ser-129 in human alpha-synuclein determines neurodegeneration in a rat model of Parkinson disease. Proc Natl Acad Sci U S A. 105:763-8
Students: Filipe Leal and Lígia Silva

Friday (Fev 1)
10:00-12:00- Paper presentation by students


Paper 3
Students: Joana Marinho and João Noutel Santos


Paper 4
* Deng HX, Shi Y, Furukawa Y, Zhai H, Fu R, Liu E, Gorrie GH, Khan MS, Hung WY, Bigio EH, Lukas T, Dal Canto MC, O´Halloran TV, Siddique T. (2006) Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A. 103:7142-7.
Students: Susana Ribeiro Louros and Ana Sofia Azevedo


Paper 5
* Bennett EJ, Shaler TA, Woodman B, Ryu KY, Zaitseva TS, Becker CH, Bates GP, Schulman H, Kopito RR. (2007) Global changes to the ubiquitin system in Huntington´s disease. Nature. 448:704-8.
Students: Sara Figueiredo and Manuel Afonso Barroso


Paper 6
* Gidalevitz T, Ben-Zvi A, Ho KH, Brignull HR, Morimoto RI. (2006) Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science. 311(5766):1471-4.
Students: Nélio Mota Gonçalves and Sueli Marques

16:00-17:00- Seminar:Protein Misfolding Disorders- Mechanisms of Protein Aggregation and Toxicity”David Borchelt

Additional Bibliography
·         Gould TW, Buss RR, Vinsant S, Prevette D, Sun W, Knudson CM, Milligan CE, Oppenheim RW. (2006) Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALSJ Neurosci. 26:8774-86..
·         Jankowsky JL, Slunt HH, Gonzales V, Savonenko AV, Wen JC, Jenkins NA, Copeland NG, Younkin LH, Lester HA, Younkin SG, Borchelt DR. (2005) Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease. PLoS Med. 2:e355.
·         Cardoso SM, Moreira PI, Agostinho P, Pereira C, Oliveira CR. (2005) Neurodegenerative pathways in Parkinson´s disease: therapeutic strategiesCurr Drug Targets CNS Neurol Disord. 4:405-19..
·         Pereira C, Agostinho P, Moreira PI, Cardoso SM, Oliveira CR. (2005) Alzheimer´s disease-associated neurotoxic mechanisms and neuroprotective strategies.4:383-403. Curr Drug Targets CNS Neurol Disord.
·         Rego AC, de Almeida LP. (2005) Molecular targets and therapeutic strategies in Huntington´s disease.4:361-81. Curr Drug Targets CNS Neurol Disord.
·         Larsen KE, Schmitz Y, Troyer MD, Mosharov E, Dietrich P, Quazi AZ, Savalle M, Nemani V, Chaudhry FA, Edwards RH, Stefanis L, Sulzer D. (2006) Alpha-synuclein overexpression in PC12 and chromaffin cells impairs catecholamine release by interfering with a late step in exocytosis. J Neurosci. 26:11915-22.
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