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: Alexander Varshavsky, Ph.D.

Friday Lecture Series

William H. Stein Memorial Lecture

Recent Discoveries about the Ubiquitin System and the N-end Rule Pathway

Alexander Varshavsky, Ph.D.,

Howard and Gwen Laurie Smits Professor of Cell Biology,

California Institute of Technology

April 26, 2012

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

Caspary Auditorium

Recommended Readings

Graciet, E., Walter, F., Maoiléidigh, D. Ó., Pollmann, S., Meyerowitz, E. M., Varshavsky, A., & Wellmer, F. (2009). The N-end rule pathway controls multiple functions during arabidopsis shoot and leaf development. Proceedings of the National Academy of Sciences of the United States of America, 106(32), 13618-13623

Hwang, C. -., Shemorry, A., Auerbach, D., & Varshavsky, A. (2010). The N-end rule pathway is mediated by a complex of the RING-type Ubr1 and HECT-type Ufd4 ubiquitin ligases. Nature Cell Biology, 12(12), 1177-1185

Hwang, C. -., Shemorry, A., & Varshavsky, A. (2010). N-terminal acetylation of cellular proteins creates specific degradation signals. Science, 327(5968), 973-977

Hwang, C. -., Shemorry, A., & Varshavsky, A. (2009). Two proteolytic pathways regulate DNA repair by cotargeting the Mgt1 alkylguanine transferase. Proceedings of the National Academy of Sciences of the United States of America, 106(7), 2142-2147

Varshavsky, A. (2006). The early history of the ubiquitin field. Protein Science, 15(3), 647-654

Varshavsky, A. (2011). The N-end rule pathway and regulation by proteolysis. Protein Science, 20(8), 1298-1345

Xia, Z., Webster, A., Du, F., Piatkov, K., Ghislain, M., & Varshavsky, A. (2008). Substrate-binding sites of UBR1, the ubiquitin ligase of the N-end rule pathway. Journal of Biological Chemistry, 283(35), 24011-24028


Protein Encyclopedia Announced by Swiss Institute of Bioinformatics

Several online resources, including ScienceDaily and EurekAlert! describe the creation of a protein encyclopedia which was announced during the 10th Anniversay Conference of the Swiss Insitute of Bioinofrmatics (SIB) in Berne, Switzerland held September 24th, 2008.  Professor Amos Bairoch, head of the SIB Swiss-Prot group described how the human genome sequencing project had created a dictionary allows a view at life as it is organized at the molecular level, noting that increasing knowledge or proteins is crucial to combating many disease states. 

The new protein encyclopedia, UniProt (Universal Protein Resource) is described as a comprehensive resource for protein sequence and annotation data.  UniProt data cover 20,325 human proteins culled from more than 45,000 scientific papers.  The information found in UniProt will provide opportunities to move closer to developing personalized medicine options for individual patients based on an understanding of their protein set.  The corpus of information will enable researchers to model the interaction between individual patients, diseases, and drugs.

Proteins from other animals will be added to the database.  The database is available for free to users.  Interestingly, in printed form, the resource would consist of 57 volumes of about 1,000 pages each.  The database can be accessed directly from the Rockefeller University Library Website.

Further details about the database are available from Janice Blondeau ( and the Swiss Institute of Bioinformatics or by contacting the Rockefeller University Library staff.