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Wan Hee Yoon

Wan Hee Yoon



Research Interests

I am interested in understanding how mis-regulation of mitochondrial metabolism and dynamics contributes to human diseases including neurodegenerative and neurodevelopmental diseases as well as aging. Using Drosophila, mouse embryonic fibroblasts and human patient fibroblasts, I discovered that Krebs cycle metabolic defects cause a progressive neurodegeneration in flies and humans.

My second research focus is to identify novel human diseases using state-of-the-art technology in Drosophila. By collaborating with human geneticists, I discovered mitochondrial genes (NRD1, OGDHL, and ATAD3A) whose mutations cause neuronal and metabolic dysfunction in flies and human. My work demonstrates that Drosophila is an excellent model system for discovering novel disease genes from genomic data of patients. Using Drosophila, I will continue to identify new human diseases, perform disease modeling in flies, and understand the mechanisms of their pathogenesis.


Publications

Yoon WH, Sandoval H, Nagarkar-Jaiswal S, Jaiswal M, Yamamoto S, Haelterman NA, Putluri N, Putluri V, Sreekumar A, Tos T, Aksoy A, Donti T, Graham BH, Ohno M, Nishi E, Hunter J, Muzny DM, Carmichael J, Shen J, Arboleda VA, Nelson SF, Wangler MF, Karaca E, Lupski JR, Bellen HJ  (2016) Loss of Nardilysin, a mitochondrial co-chaperone for α-Ketoglutarate Dehydrogenase, promotes mTORC1 activation and neurodegeneration. Neuron 93:115-131.  [Abstract]
Harel T*, Yoon WH*, Garone C, Gu S, Coban-Akdemir Z, Eldomery MK, Posey JE, Jhangiani SN, Rosenfeld JA, Cho MT, Fox S, Withers M, Brooks SM, Chiang T, Duraine L, Erdin S, Yuan B, Shao Y, Moussallem E, Lamperti C, Donati MA, Smith JD, McLaughlin HM, Eng CM, Walkiewicz M, Xia F, Pippucci T, Magini P, Seri M, Zeviani M, Hirano M, Hunter JV, Srour M, Zanigni S, Lewis RA, Muzny DM, Lotze TE, Boerwinkle E, Baylor-Hopkins Center for Mendelian Genomics, University of Washington Center for Mendelian Genomics, Gibbs RA, Hickey SE, Graham BH, Yang Y, Buhas D, Martin DM, Potocki L, Graziano C, Bellen HJ, Lupski JR (2016) Recurrent de novo and biallelic variation of ATAD3A, encoding a mitochondrial membrane protein, results in distinct neurological syndromes. American Journal of Human Genetics 99:831-845. (*equal contribution) [Abstract]
Neuronal overexpression of human VAPB slows motor impairment and neuromuscular denervation in a mouse model of ALS (2016) Kim JY, Jang A, Reddy R, Yoon WH, Jankowsky JL. Human Molecular Genetics :in press.  [Abstract]
Wong CO, Chen K, Lin YQ, Chao Y, Duraine L, Lu Z, Yoon WH, Sullivan JM, Broadhead GT, Sumner CJ, Lloyd TE, Macleod GT, Bellen HJ, Venkatachalam K (2014) A TRPV channel in Drosophila motor neurons regulates presynaptic resting Ca2+ levels, synapse growth, and synaptic transmission. Neuron 84:764-777.  [Abstract]
Haelterman NA, Yoon WH, Sandoval H, Jaiswal M, Shulman JM, Bellen HJ (2014) A mitocentric view of Parkinson's disease. Annual Review of Neuroscience 37:137-159.  [Abstract]
Montell DJ, Yoon WH, Starz-Gaiano M (2012) Group choreography: mechanisms orchestrating the collective movement of border cells. Nature Reviews Molecular Cell Biology 13:631-645.  [Abstract]
Yoon WH, Meinhardt H, Montell DJ (2011) miRNA-mediated feedback inhibition of JAK/STAT morphogen signalling establishes a cell fate threshold. Nature Cell Biology 13:1062-1069.  [Abstract]
Amanchy R, Kandasamy K, Mathivanan S, Periaswamy B, Reddy R, Yoon WH, Joore J, Beer MA, Cope L, Pandey A (2011) Identification of novel phosphorylation motifs through an integrative computational and experimental analysis of the human phosphoproteome . Journal of Proteomics and Bioinformatics 4:22-35.  [Abstract]
Hong SY*, Yoon WH*, Park JH, Kang SG, Ahn JH, Lee TH (2000) Involvement of two NF-κB binding elements in tumor necrosis factor alpha -, CD40-, and epstein-barr virus latent membrane protein 1-mediated induction of the cellular inhibitor of apoptosis protein 2 gene. Journal of Biological Chemistry 275:18022-18028. (*equal contribution) [Abstract]