Investigator: Jong-Won Lim PhD
Category: Research - Basic
FSH Research Scholar
My long-term research interest is to understand how gene expression is spatially and temporally regulated to determine cell fate and form higher structures/organs in development. For my PhD training I chose the field of Developmental Biology. My goal in Tapscott/Filippova lab is also closely related to my research interest: to define how 4qD4Z4 locus is epigenetically silenced during development and why this locus is not silenced and DUX4, the gene embedded in 4qD4Z4, is expressed in muscle cells under Facioscapulohumeral muscular dystrophy (FSHD) condition. I believe better understanding of basic sciences underlying FSHD pathogenesis will help us to develop more efficient therapeutic strategies for FSHD treatment.
My Ph.D. was carried out in the laboratory of Dr. Kristen L. Kroll at Washington University in St. Louis. As a graduate student, I defined downstream target genes and their gene network regulated by one of key neuronal genes, Neurogenin in early development. Then, I discovered a small nuclear protein, Geminin, that restrains mesodermal, endodermal, and non-neural ectodermal cell fates in early development by cooperating with Polycomb proteins. From several years in Kroll lab, I earned experience and expertise in gene expression analysis in early developmental stages.
I decided to join the research group of Dr. Gala Filippova because I would like to apply my expertise in developmental biology to human disease research. Previous studies showed DUX4 (one of strong candidate genes causing FSHD) is expressed in only ES cells and germline cells. These data suggest mechanism of DUX4 gene silencing would be closely related with developmental process. However, detailed silencing mechanism is still unclear and FSHD treatment for cure is not available yet. In Tapscott/Filipplova lab, I have earned more experience in muscle differentiation and human ES/iPS cell biology. Given my previous and current research experience in gene regulation and early development, I am well qualified to study epigenetic regulation of 4qD4Z4 units in developmental process.
Progress Report 1 (Layman Report)
Progress Report 2
Publication: DICER/AGO-dependent epigenetic silencing of D4Z4 repeats enhanced by exogenous siRNA suggests mechanisms and therapies for FSHD
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