Janghoo Lim, Ph.D., Post-doc

Phone: 713-798-7827
E-mail: jlim@bcm.edu

Joined the lab: September 2004

Project: The molecular genetic basis of the neurodegenerative diseases causing “Ataxia”.

Many human inherited cerebellar ataxias and several ataxic mouse mutants share clinical and pathological features such as loss of balance and coordination due to Purkinje cell degeneration. We developed an interaction network for proteins involved in inherited cerebellar ataxias using high-throughput yeast two-hybrid system. We found that shared molecular pathways may explain the phenotypic similarities. From here, I am working on two directions. First, what are the common underlying mechanisms for many different ataxias? Second, how Ataxin-1 interacting proteins contribute to the spinocerebellar ataxia type I (SCA1).

Selected publication:

• Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, and Zoghbi HY. Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature. 2008, 452, 713-718.
  
• Lim J, Hao T, Shaw C, Patel AJ, Szabó G, Rual J-F, Fisk CJ, Li N, Smolyar A, Hill DE, Barabási A-L, Vidal M, and Zoghbi HY. A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. Cell. 2006, 125, 801-814.