1f
12
9
1a
8
20
8
49
16
8
19
1
49
2
8
1b
1d
18
79
2b
1d
2 29
1d
25
2b
Yina Dong, PhD
4eResearch Assistant Professor of Neurology
7
60
Department: Neurology
4
1
b
1d
46
Contact information
37
4
3
23
37
Room 516E
20 Abramson Research Center
3b 34th & Civic Center Blvd
Philadelphia, PA 19104
26
20 Abramson Research Center
3b 34th & Civic Center Blvd
Philadelphia, PA 19104
2d
Office: 267-4250164
3d Lab: 215-5901451
18
7d
3d Lab: 215-5901451
18
Email:
DONGY@chop.edu
12
DONGY@chop.edu
1f
Graduate Group Affiliations
8
3
3
2
29
4
b
1f
-
64
- Neuroscience e
13
Education:
21 7 MS 17 (Physiology) c
4b Neuroscience Research Institute, Peking University, 1994.
21 8 PhD 17 (Physiology) c
4b Neuroscience Research Institute, Peking University, 1997.
c
3
3
3
3
8b
Permanent link21 7 MS 17 (Physiology) c
4b Neuroscience Research Institute, Peking University, 1994.
21 8 PhD 17 (Physiology) c
4b Neuroscience Research Institute, Peking University, 1997.
c
2 29
21
1e
1d
24
5e
8c Protein-protein interaction, Frataxin function, mitochondrial biology and metabolism, pathogenesis of cardiomyopathy, NMDA receptor
8
6b Keywords: Friedreich's ataxia, frataxin, mitochondrial dysfunction, cardiomyopathy, NMDA receptor
8
1f Description of Research
365 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.
8
1b Research Techniques
8b Cell and molecular biology, protein biochemistry, cell culture, immunohistochemistry and immunocytochemistry, transgenic mouse model
26 29
27
Description of Research Expertise
23 Research interests8c Protein-protein interaction, Frataxin function, mitochondrial biology and metabolism, pathogenesis of cardiomyopathy, NMDA receptor
8
6b Keywords: Friedreich's ataxia, frataxin, mitochondrial dysfunction, cardiomyopathy, NMDA receptor
8
1f Description of Research
365 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.
8
1b Research Techniques
8b Cell and molecular biology, protein biochemistry, cell culture, immunohistochemistry and immunocytochemistry, transgenic mouse model
26 29
23
147 Dong Y* and Watson DJ*: Thyrotropin-releasing hormone protects hippocampal neurons against glutamate toxicity via phosphatidylinositol 3-kinase/AKT pathway and new protein synthesis. bioRxiv Page: 11.11.687668, November 2025 Notes: *Corresponding author.
118 Dong Y*, Chandran V, Soragni E, Lynch DR: Editorial: The mechanistic investigation and emerging therapies for Friedreich’s ataxia. Frontiers in Pharmacology 16, February 2025 Notes: *Corresponding author.
107 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 Notes: *Corresponding author.
100 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 49 15: 352311, March 2024 Notes: *Corresponding author.
130 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.
131 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.
132 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 7a cellular and animal models of Friedreich Ataxia. Neurobiology of Disease 148(1): 105162, Jan 2021.
12e 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 56 smooth muscle cells. Scientific Reports 11(1): 8205, Apr 2021.
122 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.
de Dong YN, Lin H, Rattelle A, Panzer J and Lynch DR.: Excitotoxicity. Comprehensive toxicology (3rd edition). Elsevier Science, 6(1): 70-100, Dec 2017.
d4 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 4f receptors. Nature Communications 8(1): 15220, May 2017.
f8 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.
146 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.
121 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.
110 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.
2c
7
1d
1f
Selected Publications
140 Dong Y*, Adeshina MW, Smith R, Ngaba LV, An J, Seline O, Coulman J, Ali SAH, Mesaros C, Xu P, Lynch DR: Ketosis rescues frataxin deficiency and corrects disease phenotypes in an FRDA animal model. bioRxiv January 2026 Notes: *Corresponding author.147 Dong Y* and Watson DJ*: Thyrotropin-releasing hormone protects hippocampal neurons against glutamate toxicity via phosphatidylinositol 3-kinase/AKT pathway and new protein synthesis. bioRxiv Page: 11.11.687668, November 2025 Notes: *Corresponding author.
118 Dong Y*, Chandran V, Soragni E, Lynch DR: Editorial: The mechanistic investigation and emerging therapies for Friedreich’s ataxia. Frontiers in Pharmacology 16, February 2025 Notes: *Corresponding author.
107 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 Notes: *Corresponding author.
100 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 49 15: 352311, March 2024 Notes: *Corresponding author.
130 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.
131 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.
132 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 7a cellular and animal models of Friedreich Ataxia. Neurobiology of Disease 148(1): 105162, Jan 2021.
12e 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 56 smooth muscle cells. Scientific Reports 11(1): 8205, Apr 2021.
122 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.
de Dong YN, Lin H, Rattelle A, Panzer J and Lynch DR.: Excitotoxicity. Comprehensive toxicology (3rd edition). Elsevier Science, 6(1): 70-100, Dec 2017.
d4 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 4f receptors. Nature Communications 8(1): 15220, May 2017.
f8 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.
146 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.
121 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.
110 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.
2c