Faculty

Yina Dong, PhD

faculty photo
Research Assistant Professor of Neurology
Department: Neurology
Graduate Group Affiliations

Contact information
Room 516E
Abramson Research Center
34th & Civic Center Blvd
Philadelphia, PA 19104
Office: 267-4250164
Lab: 215-5901451
Education:
MS (Physiology)
Neuroscience Research Institute, Peking University, 1994.
PhD (Physiology)
Neuroscience Research Institute, Peking University, 1997.
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Description of Research Expertise

Research interests
Protein-protein interaction, Frataxin function, mitochondrial biology and metabolism, pathogenesis of cardiomyopathy, NMDA receptor

Keywords: Friedreich's ataxia, frataxin, mitochondrial dysfunction, cardiomyopathy, NMDA receptor

Description of Research
Dr. Dong's research focuses on the molecular basis of Friedreich's ataxia (FRDA) and its treatment options. While the etiology of FRDA is well understood, the disease's precise pathophysiology is less well defined, particularly with regard to the pathogenesis of cardiomyopathy, which results in the heart failure and mortality in FRDA patients. Dr. Dong uses cellular, molecular and proteomic approaches to investigate the function of disease-causing protein frataxin, its regulation and how its deficiency causes cellular and molecular alterations in FRDA models. Her work demonstrated a direct link between frataxin and ketone body metabolism, and also unveils new revenues for therapy development. In addition to exploring the molecular mechanism behind the pathophysiology of FRDA, Dr. Dong is also interested in the function of peripheral NMDA receptors.

Research Techniques
Cell and molecular biology, protein biochemistry, cell culture, immunohistochemistry and immunocytochemistry, transgenic mouse model

Selected Publications

Dong YN*, Mercado-Ayón E, Coulman J, Flatley L, Ngaba LV, Adeshina MW, Lynch DR : The Regulation of the Disease-Causing Gene FXN. Cells 13(12): 1040, June 2024.

Dong YN*, Ngaba LV, An J, Adeshina MW, Warren N, Wong J, Lynch DR*: A peptide derived from TID1S rescues frataxin deficiency and mitochondrial defects in FRDA cellular models. Frontiers in Pharmacology 15: 352311, March 2024 Notes: *Corresponding author.

Dong YN, Mesaros C, Xu P, Mercado-Ayon E, Halawani S, Ngaba L, Warren N, Sleiman P, Rodden L, Schadt KA, Blair IA, Lynch DR: Frataxin controls ketone body metabolism through regulation of OXCT1. PNAS Nexus 1(3): pgac 142, Jul 2022.

Rodden LN, Rummey C, Dong YN and Lynch DR: Clinical evidence for variegated silence in Friedreich ataxia patients: Ceiling effect of GAA repeat length reveals a clinical homogeneous subgroup. Neurology Genetics 8(3): 1683, May 2022.

Rodden LN, Rummey C, Dong YN, Lagedrost S, Regner S, Brocht A, Bushara K, Delatycki MB, Gomez C, Mathews K, Murray S, Perlman S, Ravina B, Subramony SH, Wilmot G, Zesiewicz T, Bolotta A, Dommisy A, Jespersen C, Ji B, Soragni E, Gottesfeld J, Lynch DR: A non-synonymous SNP in SIRT6 predicts neurological severity in Friedreich ataxia. Frontiers in Molecular Biosciences 9(1): 922788, Sept 2022.

Rodríguez-Pascau L, Britti E, Calap-Quintana P, Dong YN, Vergara C, Delaspre F, Medina-Carbonero M, Tamarit J, Pallardó FV, Gonzalez-Cabo P, Ros J, Lynch DR, Martinell M, Pizcueta P: PPAR gamma agonist leriglitazone improves frataxin-loss impairments in cellular and animal models of Friedreich Ataxia. Neurobiology of Disease 148(1): 105162, Jan 2021.

Dong YN, Hsu FC, Koziol-White CJ, Stepanova V, Jude J, Gritsiuta A, Rue R, Mott R, Coulter DA, Panettieri RA Jr, Krymskaya VP, Takano H, Goncharova EA, Goncharov DA, Cines DB, Lynch DR: Functional NMDA receptors are expressed by human pulmonary artery smooth muscle cells. Scientific Reports 11(1): 8205, Apr 2021.

Dong YN, McMillan E, Clark EM, Lin H, Lynch DR: GRP75 overexpression rescues frataxin deficiency and mitochondrial phenotypes in Friedreich ataxia cellular models. Human Molecular Genetics 28(10): 1594-1607, May 2019.

Scanlon DP, Bah A, Krzeminski M, Zhang W, Leduc-Pessah HL, Dong YN, Forman-Kay JD, Salter MW: An evolutionary switch in ND2 enables Src kinase regulation of NMDA receptors. Nature Communications 8(1): 15220, May 2017.

Dong YN, Lin H, Rattelle A, Panzer J and Lynch DR.: Excitotoxicity. Comprehensive toxicology (3rd edition). Elsevier Science, 6(1): 70-100, Dec 2017.

Salter MW, Dong YN, Kalia LV, Liu XJ and Pitcher G: Regulation of NMDA receptors by kinases and phosphatases. Biology of the NMDA Receptor. CRC Press, Page: 123-148, Oct 2008.

Liu XJ, Gingrich JR, Vargas-Caballero M, Dong YN, Sengar A, Beggs S, Wang SH, Ding HK, Frankland PW, Salter MW: Treatment of inflammatory and neuropathic pain by uncoupling Src from the NMDA receptor complex. Nature Medicine 14(12): 1325-1332, Dec 2008.

Dong YN, Waxman EA and Lynch DR: Interactions of postsynaptic density-95 and the NMDA receptor 2 subunit control calpain-mediated cleavage of the NMDA receptor. The Journal of Neuroscience 24(49): 11035-45, Dec 2004.

Simpkins K, Guttmann RP, Dong Y, Chen Z, Sokol S, Neumar RW, Lynch DR: Selective activation induced cleavage of the NR2B subunit by calpain. The Journal of Neuroscience 23(36): 11322-11331, Dec 2003.

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Last updated: 06/24/2024
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