Yale Genetics

Our Department Faculty/Labs Graduate Program Medical Genetics Directory

    Lim, Janghoo

    Assistant Professor of Genetics and Program in Cellular Neuroscience, Neurodegeneration and Repair

    * B.S. Chonnam National University, Korea, 1997

    * M.S. Korea Advanced Institute of Science and Technology, Korea, 1999

    * Ph.D. Baylor College of Medicine, 2004

    Honors:

    * Pathway to Independence Award, NIH/NINDS, 2009

    Research Interests:

    * Mechanisms of Neurodegenerative diseases

    * Neural development and neurodevelopmental disorders

    Current Research:

    Neurodegenerative disease affects over six million Americans and the burden of these devastating disorders is enormous.  Despite decades of research in a variety of model organisms, the pathogenic mechanisms underlying most of these debilitating disorders are not well understood.

    The main interest in our laboratory is to understand the molecular basis of cellular pathology in neurodegenerative diseases and ultimately to translate the findings into the development of therapeutics to treat neurodegeneration.  Our laboratory uses the combined approaches of biochemistry, cell biology, behavior, and molecular genetics with in vivo animal models to explore the pathogenesis of diverse neurodegenerative diseases.

    We are particularly interested in a protein called Nemo-like kinase (NLK) because it is likely to play a role in more than one neurodegenerative disease and because NLK is an evolutionarily conserved serine/threonine kinase whose activity could be modulated to exploit it as a therapeutic target.  Our laboratory has generated Nlk knock-out and overexpression models in mice as well as in Drosophila.  We are studying the role of NLK (and its signal transduction pathways) in the pathogenesis of diverse neurodegenerative diseases.  We are also beginning to study the function of NLK in neural development, and how NLK activity contributes to the pathogenesis of neurodevelopmental disorders.

    Representative Publications

    Representative Publications

    Lim, J., Crespo-Barreto, J., Jafar-Nejad, P., Bowman, A.B., Richman, R., Hill, D.E., Orr, H.T., and Zoghbi, H.Y. (2008). Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature 452, 713-718.

    Al-Ramahi, I., Pérez, A.M., Lim, J., Zhang, M., Sorensen, R., de Haro, M., Branco, J., Pulst, S.M., Zoghbi, H.Y., and Botas, J. (2007). dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet. 3, e234.

    Lam, Y.C., Bowman, A.B., Jafar-Nejad, P., Lim, J., Richman, R., Fryer, J.D., Hyun, E.D., Duvick, L.A., Orr, H.T., Botas, J., and Zoghbi, H.Y. (2006). ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell 127, 1335-1347.

    Lim, J., Hao, T., Shaw, C., Patel, A.J., Szabó, G., Rual, J.F., Fisk, C.J., Li, N., Smolyar, A., Hill, D.E., Barabási, A.L., Vidal, M., and Zoghbi, H.Y. (2006). A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. Cell 125, 801-814.
    Our Department Faculty/Labs Graduate Program Medical Genetics Directory