Seung-Beom Hong, Ph.D.

Research Associate
University of Pennsylvania Medical Center
Pulmonary, Allergy, & Critical Care Division
Translational Research Laboratories (TRL)
125 South 31st Street, TRL Suite 1200
Philadelphia, PA 19104-3413

tel.: (215) 573-9936
fax: (215) 746-1224

Education: Ph.D., Korea Advanced Institute of Science and Technology (KAIST), Daejeon, Republic of Korea
M.S., KAIST, Daejeon, Republic of Korea.

Postdoctoral Fellowship: University of Pennsylvania , Philadelphia, PA

Dr. Hong joined the Airways Biology Initiative (ABI) in 2011 as a research associate in Dr. Krymskaya's laboratory. He is currently studying Birt-Hogg-Dube'(BHD) syndrome, which is characterized by the development of fibrofolliculomas, lung cysts and renal carcinoma and caused by germ line mutations in the folliculin (FLCN) gene. FLCN forms a complex with novel folliculin-interacting proteins 1 and 2 (FNIP1 and FNIP2), and 5'-AMP-activated protein kinase (AMPK), an energy sensing kinase that is activated in energetic stress condition. He has identified that FLCN inactivation induces transcriptional activity of an oncogenic transcription factor TFE3 by increasing its nuclear localization. Nuclear localization of TFE3 was associated with post-translational modifications of TFE3 including decreased phosphorylation. Dr. Krymskaya's laboratory is trying to better understand the mechanism of how FLCN inactivation leads to the activation of TFE3and the biological processes it regulates. They have shown that the TFE3 transcription factor plays a role in the regulation of cell-cell or cell-matrix contact using an in vitro system. They are examining the possible role of TFE3 in energy metabolism in connection with AMPK and energetic stresses and are investigating the importance of TFE3 and AMPK in the development of BHD syndrome using cancer cell and mouse models. They are also investigating the possibility that TFE3 and AMPK can be used as therapeutic targets for BHD syndrome.

Selected Publications:

  1. Hong SB, Oh HB, Valera VA, Baba M, Schmidt LS and Linehan WM. "Inactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localization”. PLoS One. 2010 Dec 29;5(12)
  2. Hong SB, Oh H, Valera VA, Stull J, Ngo DT, Baba M, Merino MJ, Linehan WM, Schmidt LS. "Tumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-beta signaling.” Mol Cancer 9(1):160, 2010
  3. Hasumi Y, Baba M, Ajima R, Hasumi H, Valera VA, Klein ME, Haines DC, Merino MJ, Hong SB, Yamaguchi TP, Schmidt LS, Linehan WM. Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2. Proc Natl Acad Sci U S A. 106(44):18722-7, 2009
  4. Hasumi H, Baba M, Hong SB, Hasumi Y, Huang Y, Yao M, Valera VA, Linehan WM, Schmidt LS. Identification and characterization of a novel folliculin-interacting protein FNIP2. Gene. 415 (1-2): 60-67, 2008
  5. Baba M, Furihata M, Hong SB, Tessarollo L, Haines DC, Southon E, Patel V, Igarashi P, Alvord WG, Leighty R, Yao M, Bernardo M, Ileva L, Choyke P, Warren MB, Zbar B, Linehan WM, Schmidt LS. Kidney-Targeted Birt-Hogg-DubÉ Gene Inactivation in a Mouse Model: Erk1/2 and Akt-mTOR Activation, Cell Hyper-proliferation, and Polycystic Kidneys. J Natl Cancer Inst.100(2):140-54, 2008
  6. Baba M, Hong SB, Sharma N, Warren MB, Nickerson ML, Iwamatsu A, Esposito D, Gillette WK, Hopkins RF 3rd, Hartley JL, Furihata M, Oishi S, Zhen W, Burke TR Jr, Linehan WM, Schmidt LS, Zbar B. Folliculin encoded by the BHD gene interacts with a novel binding protein, FNIP1, and AMPK, and is involved in AMPK and mTOR signaling. Proc Natl Acad Sci U S A. 103: 15552-15557, 2006
  7. Hong SB, Furihata M, Baba M, Zbar B, Schmidt LS. Vascular defects and liver damage by the acute inactivation of the VHL gene during mouse embryogenesis. Laboratory Investigation 86: 664-75, 2006