Neurodegenerative and neurodevelopmental diseases affect millions of people around the globe. Scientists have thought many of these diseases may result from disruptions or failures in the mitochondria, a specialized cellular structure often referred to as the “powerhouse of the cell.”
Mitochondria play a key role in metabolism in cells. Metabolism is a series of biochemical reactions essential for all living organisms. It provides energy and the building blocks for healthy cells. As we age, we lose some of these capabilities of mitochondria, and the quality of our cells declines. This has been implicated in many diseases, including neurodegenerative diseases like Alzheimer’s and Parkinson’s and even cancer.
Our long-term goal is to learn how cells maintain mitochondrial structure and metabolism, how failures in that system may contribute to human pathologies and, ultimately, to find treatments for those diseases.
To do this, we use common fruit flies called Drosophila as a disease model. Because fruit flies share approximately 75 percent of a human’s disease genes, they are near-perfect tools for studying conditions that afflict people. We “humanize” these flies by using state-of-the-art gene editing tools. By studying how their mitochondrial dysfunction contributes to defects in flies, we can get hints into the root causes for human diseases.
My lab also focuses on identifying novel and previously unknown human diseases using the newest technologies in Drosophila. In collaboration with human geneticists, I discovered mitochondrial genes whose mutations cause neuronal and metabolic dysfunction in both flies and humans. This work shows that fruit flies serve as an excellent model system for discovering novel disease genes from human patient data. Using these tiny flies, we can continue to identify new human diseases, perform disease modeling in flies, and understand the mechanisms of their pathogenesis.
B.Sc., Yonsei University, South Korea, 1997
M. Sc., Yonsei University, South Korea, 1999
Ph.D., Johns Hopkins University School of Medicine, 2011
2016-2017 Assistant Professor, Department of Molecular and Human Genetics, Baylor College of Medicine
2011-2016 Postdoctoral Research Fellow, Howard Hughes Medical Institute, Department of Molecular and Human Genetics, Baylor College of Medicine Principal Investigator: Hugo J. Bellen, D.V.M., Ph.D.
1999-2004 Senior Research Scientist, Central Research Institute, Choongwae Pharmaceutical Company, South Korea
2001 Visiting Research Scientist, Pacific Northwest Diabetes Research Institute, Seattle. Principal Investigator: Michael Kahn, Ph.D.
Honors and Awards
2004 Korea Science and Engineering Foundation Fellowship
2005 H.A. and Mary K. Chapman Young Investigator Fellowship
2015 Best Oral Presentation Award, 14th Korean-American Biomedical Scientists Symposium
2016 Oral Presentation Award, 15th Korean-American Biomedical Scientists Symposium
2017 Best Postdoctoral Publication Award, Department of Molecular and Human Genetics, Baylor College of Medicine
Joined OMRF Scientific Staff in 2017
Oláhová M*, Yoon WH*, Thompson K*, Jangam S, Fernandez-Betancourt, EL , Davidson JM, Kyle JE, Grove ME, Fisk DG, Kohler J, Holmes M, Dries AM, Huang Y, Zhao C, Contrepois K, Zappala Z, Frésard L, Waggott D, Zink EM, Kim YM, Heyman HM, Stratton KG, Webb-Robertson BM, Undiagnosed Diseases Network, Snyder M, Merker JD, Montgomery SB, Fisher PG, Feichtinger RG, Mayr JA, Hall J, Barbosa IA, Simpson MA, Deshpande C, Waters KM, Koeller D, Metz TO, Morris AA, McFarland R, Schelley S, Van Hove JLK, Enns GM, Yamamoto S, Ashley EA, Wangler MF, Taylor RW, Bellen HJ, Bernstein JA, Wheeler MT. Bi-allelic mutations in ATP5F1D, a subunit of ATP synthase, cause a metabolic disorder. Am J Hum Genet. 2018 Mar 1;102(3):494-504. *equal contribution PMID: 29478781
Yoon WH, Sandoval H, Nagarkar-Jaiswal S, et al. Loss of Nardilysin, a mitochondrial co-chaperone for α-Ketoglutarate dehydrogenase, promotes mTORC1 activation and neurodegeneration. Neuron. 2017 Jan 4;93(1):115-31. PMCID: PMC5242142
- Highlighted in the following:
EurekAlert! (2016) “Genes Nardilysin and OGDHL linked to human neurological condition”
News release, Baylor College of Medicine (2016) “Genes Nardilysin and OGDHL linked to human neurological condition”
News release, Jan and Dan Duncan Neurological Research Institute (2016) “Mutations in Nardilysin and OGDHL causes neurodegeneration”
Harel T*, Yoon WH*, Garone C, et al. Recurrent de novo and biallelic variation of ATAD3A, encoding a mitochondrial membrane protein, results in distinct neurological syndromes. Am J Hum Genet. 2016 Oct 6;99(4):831-45. PMCID: PMC5065660 *equal contribution
- Highlighted in the followings
EurekAlert! (2016) “Distinct neurological syndromes can be the result of variations in gene ATAD3A”
News release, Baylor College of Medicine (2016) “Distinct neurological syndromes can be the result of variations in gene ATAD3A”
News Medical Life Sciences (2016) “Rare neurological syndromes linked to variantions in ATAD3A gene”
Wong CO, Chen K, Lin YQ, Chao Y, Duraine L, Lu Z, Yoon WH, et al. A TRPV channel in Drosophila motor neurons regulates presynaptic resting Ca2+ levels, synapse growth, and synaptic transmission. Neuron. 2014 Nov 19;84(4):764-77. PMCID: PMC4254599
Haelterman NA, Yoon WH, Sandoval H, Jaiswal M, Shulman JM, Bellen HJ. A mitocentric view of Parkinson's disease. Annu Rev Neurosci. 2014;37:137-59. Review. PMCID: PMC4659514
Montell DJ, Yoon WH, Starz-Gaiano M. Group choreography: mechanisms orchestrating the collective movement of border cells. Nat Rev Mol Cell Biol. 2012 Oct;13(10):631-45. PMCID: PMC4099007
Yoon WH, Meinhardt H, Montell DJ. miRNA-mediated feedback inhibition of JAK/STAT morphogen signalling establishes a cell fate threshold. Nat Cell Biol. 2011 Aug 21;13(9):1062-9. PMCID: PMC3167036
Functional & Chemical Genomics Research Program, MS 46
Oklahoma Medical Research Foundation
825 N.E. 13th Street
Oklahoma City, OK 73104
Phone: (405) 271-1574
Fax: (405) 271-3765