Recommended Readings: Susan L. Ackerman, Ph.D. May 2

Friday Lecture Series
Friday, April 25, 2014
3:45 p.m., Caspary Auditorium

Susan L. Ackerman, Ph.D.
Professor, The Jackson Laboratory
Investigator, Howard Hughes Medical Institute

Multicopy Genes Encoding Functional RNAs and Their Role in the CNS

Recommended Readings:

Empirical Articles

Jia, Y., Mu, J. C., & Ackerman, S. L. (2012). Mutation of a U2 snRNA gene causes global disruption of alternative splicing and neurodegeneration. Cell, 148(1-2), 296–308. doi:10.1016/j.cell.2011.11.057

Liu, Y., Zaun, H. C., Orlowski, J., & Ackerman, S. L. (2013). CHP1-mediated NHE1 biosynthetic maturation is required for Purkinje cell axon homeostasis. The Journal of Neuroscience, 33(31), 12656–12669. doi:10.1523/JNEUROSCI.0406-13.2013

Zhao, L., Longo-Guess, C., Harris, B. S., Lee, J.-W., & Ackerman, S. L. (2005). Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP. Nature Genetics, 37(9), 974–979. doi:10.1038/ng1620

Review Papers

Cooper, T. A, Wan, L., & Dreyfuss, G. (2009). RNA and disease. Cell, 136(4), 777–793. doi:10.1016/j.cell.2009.02.011

Lemmens, R., Moore, M. J., Al-Chalabi, A., Brown, R. H., & Robberecht, W. (2010). RNA metabolism and the pathogenesis of motor neuron diseases. Trends in Neurosciences, 33(5), 249–258. doi:10.1016/j.tins.2010.02.003

Recommended Readings: Huda Zoghbi, M.D.

Friday Lecture Series

Neurodegenerative Diseases: The Dangers of Too Much Protein Stability

Huda Zoghbi, M.D., professor, Baylor College of Medicine;

investigator, Howard Hughes Medical Institute;

director, Jan and Dan Duncan Neurological Research Institute,

Texas Children’s Hospital

December 6, 2013

3:45 p.m.-5:00 p.m. (Refreshments, 3:15 p.m., Abby Lounge)

Caspary Auditorium

Recommended Readings

Chahrour, M., & Zoghbi, H. Y. (2007). The story of rett syndrome: From clinic to neurobiology. Neuron, 56(3), 422-437

Fernandez-Funez, P., Nino-Rosales, M. L., De Gouyon, B., She, W. -., Luchak, J. M., Martinez, P., . . . Botas, J. (2000). Identification of genes that modify ataxin-1-induced neurodegeneration. Nature, 408(6808), 101-106

Jafar-Nejad, P., Ward, C. S., Richman, R., Orr, H. T., & Zoghbi, H. Y. (2011). Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America, 108(5), 2142-2147

Lim, J., Hao, T., Shaw, C., Patel, A. J., Szabó, G., Rual, J. -., . . . Zoghbi, H. Y. (2006). A protein-protein interaction network for human inherited ataxias and disorders of purkinje cell degeneration. Cell, 125(4), 801-814

Riley, B. E., Zoghbi, H. Y., & Orr, H. T. (2005). SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal. Journal of Biological Chemistry, 280(23), 21942-21948

Zoghbi, H. Y., & Botas, J. (2002). Mouse and fly models of neurodegeneration. Trends in Genetics, 18(9), 463-471

Zoghbi, H. Y., & Warren, S. T. (2010). Neurogenetics: Advancing the “next-generation” of brain research. Neuron, 68(2), 165-173

 

Recommended Readings: Hugo Bellen, Ph.D., D.V.M.

Friday Lecture Series

Mitochondria and Neurodegeneration

Hugo Bellen, Ph.D., D.V.M., investigator, Howard Hughes Medical Institute;

professor, departments of molecular and human

genetics and neuroscience, director, Program in Developmental Biology,

Baylor College of Medicine

March 30, 2012

3:45 p.m.-5:00 p.m. (Refreshments, 3:15 p.m., Abby Lounge)

Caspary Auditorium

 

Recommended Readings:

Bibliography

Han, S. M., H. Tsuda, Y. Yang, J. Vibbert, P. Cottee, S. -J Lee, J. Winek, C. Haueter, H. J. Bellen, and M. A. Miller. 2012. “Secreted VAPB/ALS8 Major Sperm Protein Domains Modulate Mitochondrial Localization and Morphology Via Growth Cone Guidance Receptors.” Developmental Cell 22 (2): 348-362

Pesah, Y., T. Pham, H. Burgess, B. Middlebrooks, P. Verstreken, Y. Zhou, M. Harding, H. Bellen, and G. Mardon. 2004. “Drosophila Parkin Mutants have Decreased Mass and Cell Size and Increased Sensitivity to Oxygen Radical Stress.” Development 131 (9): 2183-2194

Romero, E., G. -H Cha, P. Verstreken, C. V. Ly, R. E. Hughes, H. J. Bellen, and J. Botas. 2008. “Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm.” Neuron 57 (1): 27-40

Venken, K. J. T., Y. He, R. A. Hoskins, and H. J. Bellen. 2006. “P[Acman]: A BAC Transgenic Platform for Targeted Insertion of Large DNA Fragments in D. Melanogaster.” Science 314 (5806): 1747-1751

Verstreken, P., C. V. Ly, K. J. T. Venken, T. -W Koh, Y. Zhou, and H. J. Bellen. 2005. “Synaptic Mitochondria are Critical for Mobilization of Reserve Pool Vesicles at Drosophila Neuromuscular Junctions.” Neuron 47 (3): 365-378


A Fresh Look At The Validity Of Current Understanding Of Alzheimer’s Disease

For decades the amyloid hypothesis has dominated the research field in Alzheimer’s disease. The theory describes how an increase in secreted beta-amyloid peptides leads to the formation of plaques, toxic clusters of damaged proteins between cells, which eventually result in neurodegeneration. Scientists at Lund University, Sweden, have now presented a study that turns this premise on its head. The research group’s data offers an opposite hypothesis, suggesting that it is in fact the neurons’ inability to secrete beta-amyloid that is at the heart of pathogenesis in Alzheimer’s disease.  Read the report of their work in Journal of Neuroscience.

New Drug Target for Alzheimer’s, Stroke Discovered

A tiny piece of a critical receptor that fuels the brain and without which sentient beings cannot live has been discovered by University at Buffalo scientists as a promising new drug target for Alzheimer’s and other neurodegenerative diseases.

The research on the NMDA (N-methyl-D-aspartate) receptor was being published online  in Nature Communications.

Scientists Spot New Clues to HIV-Linked Dementia

Researchers have identified two genetically distinct types of HIV in the cerebrospinal fluid (CSF) of patients with HIV-associated dementia.   The discovery may help explain why the risk of developing neurological difficulties increases as AIDS patients live longer, and may also help predict which patients are at greatest risk for the problem, according to the U.S. scientists.  They said the two newly-identified HIV types aren’t being detected in HIV that circulates in a patient’s blood, and one type may be present cerebrospinal fluid years before the onset of HIV-linked dementia.  The fact that the two HIV types can be detected in the CSF indicates that they grow in the central nervous system. 

The study, which appears in the journal PLoS Pathogens, was led by researchers at the University of North Carolina at Chapel Hill School of Medicine.

New Insights Into The Mechanism Of Parkinson’s Disease

New research suggests that small “seed” amounts of diseased brain proteins can be taken up by healthy neurons and propagated within them to cause neurodegeneration. The research, published by Cell Press in the October 6, 2011 issue of the journal Neuron, sheds light on the mechanisms associated with Parkinson’s disease (PD) and provides a model for discovering early intervention therapeutics that can prevent or slow the devastating loss of neurons that underlies PD.

New Mechanism for Dementia?

Hyperactive immune cells that engulf dying and injured cells before they have a chance to recover may contribute to neurodegeneration characteristic of some diseases, posing a previously unreported mechanism for dementia.  Biochemists of the University of California, San Francisco found preliminary evidence for a different pathway of neurodegeneration — one that involves the clearance of apoptotic cells by overly active phagocytic immune cells, such as macrophages, leading to widespread cell loss over time.  Read the full report of this research in PNAS.

Recommended Readings: Junying Yuan, Ph.D.

 

Special Seminar Series

A Quest to Understand the Mechanisms of Neurodegeneration

Junying Yuan, Ph.D.

Professor, Department of Cell Biology

Harvard Medical School

Wednesday, March 18, 2009

4:00 p.m.-5:00 p.m. (Refreshments, 3:45 p.m.)

Second Floor, Welch Hall

Recommended Articles:

Panaretakis T, Kepp O, Brockmeier U, Tesniere A, Bjorklund A-, Chapman DC, Durchschlag M, Joza N, Pierron G, van Endert P, Yuan J, Zitvogel L, Madeo F, Williams DB, Kroemer G. Mechanisms of pre-apoptotic calreticulin exposure in immunogenic cell death. EMBO Journal. 2009.

Yi CH, Yuan J. The jekyll and hyde functions of caspases. Developmental Cell. 2009;16(1):21-34.

Yuan J. Neuroprotective strategies targeting apoptotic and necrotic cell death for stroke. Apoptosis. 2009:1-9.
Article in press.

Hitomi J, Christofferson DE, Ng A, Yao J, Degterev A, Xavier RJ, Yuan J. Identification of a molecular signaling network that regulates a cellular necrotic cell death pathway. Cell. 2008;135(7):1311-23.

Zheng W, Degterev A, Hsu E, Yuan J, Yuan C. Structure-activity relationship study of a novel necroptosis inhibitor, necrostatin-7. Bioorganic and Medicinal Chemistry Letters. 2008;18(18):4932-5.

You Z, Savitz SI, Yang J, Degterev A, Yuan J, Cuny GD, Moskowitz MA, Whalen MJ. Necrostatin-1 reduces histopathology and improves functional outcome after controlled cortical impact in mice. Journal of Cerebral Blood Flow and Metabolism 2008;28(9):1564-73.
Contact the Markus Library for a reprint of this article.

Degterev A, Hitomi J, Germscheid M, Ch’en IL, Korkina O, Teng X, Abbott D, Cuny GD, Yuan C, Wagner G, Hedrick SM, Gerber SA, Lugovskoy A, Yuan J. Identification of RIP1 kinase as a specific cellular target of necrostatins. Nature Chemical Biology. 2008;4(5):313-21.

Cuny GD, Degterev A, Yuan J. Necroptosis – A novel cell death mechanism. Drugs of the Future 2008;33(3):225-33.
Contact the Markus Library for a reprint of this article.

Degterev A, Boyce M, Yuan J. A decade of caspases. Oncogene. 2003;22(53 REV. ISS. 7):8543-67.