|EDUCATION/TRAINING||PhD, Pharmacology, Rutgers University/UMDNJ, USA ; Post-doc, Genetics, Stanford University, USA|
|研究專長||Supt4h on Huntington's disease and other polyQ-mediated neurodegenerative disorders|
Huntington’s disease (HD) and multiple neurodegenerative disorders are caused by CAG tri-nucleotide expansion. In our earlier published investigation, we showed that the yeast transcription elongation factor Spt4 modulates the expression of genes containing expanded CAG repeats, while having marginal effects on the one with short or no CAG repeats. Lowering SUPT4H (which is homolog of yeast Spt4 in mammalian cells) can selectively reduce the mutant huntingtin gene expression, ameliorate polyQ-mediated protein aggregation, and rescue cells from the deleterious effect of CAG expansion genes in murine striatal neurons. Additional animal studies also reveal that reduction of SUPT4H in mouse brains is associated with decreased HTT protein aggregation, prolonged lifespan, and delay of the motor impairment that normally develops in HD mouse model of R6/2.
We currently are aimed to further understand the functional aspects of SUPT4H in transcription of CAG-expanded DNA template and to assess the suitability of SUPT4H as a therapeutic target against HD and other CAG expansion diseases.