ATP Synthase Function and Regulation

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Nakamura, K., Aoyama-Ishiwatari, S., Nagao, T., Paaran, M., Obara, C. J., Sakurai-Saito, Y., Johnston, J., Du, Y., Suga, S., Tsuboi, M., Nakakido, M., Tsumoto, K., Kishi, Y., Gotoh, Y., Kwak, C., Rhee, H.-W., Seo, J. K., Kosako, H., Potter, C., … Hirabayashi, Y. (2025). Mitochondrial complexity is regulated at ER-mitochondria contact sites via PDZD8-FKBP8 tethering. Nature Communications, 16(1). https://doi.org/10.1038/s41467-025-58538-3

Publication Date

2025-04-18

Columbia Affiliation

Department of Neuroscience; Vagelos College of Physicians and Surgeons

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Karaa, A., Bertini, E., Carelli, V., Cohen, B., Ennes, G. M., Falk, M. J., Goldstein, A., Gorman, G., Haas, R., Hirano, M., Klopstock, T., Koenig, M. K., Kornblum, C., Lamperti, C., Lehman, A., Longo, N., Molnar, M. J., Parikh, S., Phan, H., Pitceathly, R. D. S., … MMPOWER-3 Trial Investigators (2024). Genotype-specific effects of elamipretide in patients with primary mitochondrial myopathy: a post hoc analysis of the MMPOWER-3 trial. Orphanet journal of rare diseases, 19(1), 431. https://doi.org/10.1186/s13023-024-03421-5

Publication Date

2024-11-21

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Neurology

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Morcillo, P., Kabra, K., Velasco, K., Cordero, H., Jennings, S., Yun, T. D., Larrea, D., Akman, H. O., & Schon, E. A. (2024). Aberrant ER-mitochondria communication is a common pathomechanism in mitochondrial disease. Cell Death & Disease, 15(6). https://doi.org/10.1038/s41419-024-06781-9

Publication Date

2024-06-10

Columbia Affiliation

Institute of Human Nutrition; Vagelos College of Physicians and Surgeons; Department of Neurology; Center for Translational Immunology; Department of Genetics and Development

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Nguyen, T. T. T., Torrini, C., Shang, E., Shu, C., Mun, J.-Y., Gao, Q., Humala, N., Akman, H. O., Zhang, G., Westhoff, M.-A., Karpel-Massler, G., Bruce, J. N., Canoll, P., & Siegelin, M. D. (2024). OGDH and Bcl-xL loss causes synthetic lethality in glioblastoma. JCI Insight. https://doi.org/10.1172/jci.insight.172565

Publication Date

2024-03-14

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Pathology and Cell Biology; Department of Neurosurgery; Department of Neurology

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Cao, Q., Hajosch, A., Kast, R. E., Loehmann, C., Hlavac, M., Fischer-Posovszky, P., Strobel, H., Westhoff, M.-A., Siegelin, M. D., Wirtz, C. R., Halatsch, M.-E., & Karpel-Massler, G. (2024). Tumor Treating Fields (TTFields) combined with the drug repurposing approach CUSP9v3 induce metabolic reprogramming and synergistic anti-glioblastoma activity in vitro. British Journal of Cancer, 130(8), 1365–1376. https://doi.org/10.1038/s41416-024-02608-8

Publication Date

2024-02-23

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Pathology and Cell Biology

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Chang, Y.-H., Kang, E. Y.-C., Liu, L., Jenny, L. A., Khang, R., Seo, G. H., Lee, H., Chen, K.-J., Wu, W.-C., Hsiao, M.-C., & Wang, N.-K. (2023). Maternal mosaicism in SSBP1 causing optic atrophy with retinal degeneration: implications for genetic counseling. Orphanet Journal of Rare Diseases, 18(1). https://doi.org/10.1186/s13023-023-02748-9

Publication Date

2023-12-01

Columbia Affiliation

Department of Ophthalmology; Vagelos College of Physicians and Surgeons; Department of Pathology and Cell Biology

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Sturm, G., Karan, K. R., Monzel, A. S., Santhanam, B., Taivassalo, T., Bris, C., Ware, S. A., Cross, M., Towheed, A., Higgins-Chen, A., McManus, M. J., Cardenas, A., Lin, J., Epel, E. S., Rahman, S., Vissing, J., Grassi, B., Levine, M., Horvath, S., … Picard, M. (2023). OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases. Communications Biology, 6(1). https://doi.org/10.1038/s42003-022-04303-x

Publication Date

2023-12-01

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Psychiatry; Division of Behavioral Medicine; Department of Systems Biology; Department of Neurology; Columbia Translational Neuroscience Initiative; H. Houston Merritt Center for Neuromuscular and Mitochondrial Disorders; Department of Biochemistry and Molecular Biophysics; Department of Medicine; Division of General Medicine; Center of Excellence for Sleep and Circadian Research

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Murari, A., Rhooms, S.-K., Vimal, D., Hossain, K. F. B., Saini, S., Villanueva, M., Schlame, M., & Owusu-Ansah, E. (2023). Phospholipids can regulate complex I assembly independent of their role in maintaining mitochondrial membrane integrity. Cell Reports, 42(8), 112846. https://doi.org/10.1016/j.celrep.2023.112846

Publication Date

2023-08-01

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Physiology and Cellular Biophysics

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Fernandez Garcia, E., Paudel, U., Noji, M. C., Bowman, C. E., Rustgi, A. K., Pitarresi, J. R., Wellen, K. E., Arany, Z., Weissenrieder, J. S., & Foskett, J. K. (2023). The mitochondrial Ca2+ channel MCU is critical for tumor growth by supporting cell cycle progression and proliferation. Frontiers in Cell and Developmental Biology, 11. https://doi.org/10.3389/fcell.2023.1082213

Publication Date

2023-07-06

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Medicine; Division of Digestive and Liver Diseases; Herbert Irving Comprehensive Cancer Center

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Menale, C., Trinchese, G., Aiello, I., Scalia, G., Dentice, M., Mollica, M. P., Yoon, N. A., & Diano, S. (2023). Nutrient-Dependent Mitochondrial Fission Enhances Osteoblast Function. Nutrients, 15(9), 2222. https://doi.org/10.3390/nu15092222

Publication Date

2023-05-08

Columbia Affiliation

Institute of Human Nutrition; Vagelos College of Physicians and Surgeons; Department of Molecular Pharmacology and Therapeutics; Department of Physiology and Cellular Biophysics

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Padavannil, A., Murari, A., Rhooms, S.-K., Owusu-Ansah, E., & Letts, J. A. (2023). Resting mitochondrial complex I from Drosophila melanogaster adopts a helix-locked state. ELife, 12. CLOCKSS. https://doi.org/10.7554/elife.84415

Publication Date

2023-03-23

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Physiology and Cellular Biophysics; Robert N. Butler Columbia Aging Center

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Mnatsakanyan, N., Park, H.-A., Wu, J., He, X., Llaguno, M. C., Latta, M., Miranda, P., Murtishi, B., Graham, M., Weber, J., Levy, R. J., Pavlov, E. V., & Jonas, E. A. (2022). Mitochondrial ATP synthase c-subunit leak channel triggers cell death upon loss of its F1 subcomplex. Cell Death & Differentiation, 29(9), 1874–1887. https://doi.org/10.1038/s41418-022-00972-7

Publication Date

2022-09-01

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Anesthesiology

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Barua, S., Berger, S., Pereira, E. M., & Jobanputra, V. (2022). Expanding the phenotype of ATP6AP1 deficiency. Molecular Case Studies, 8(4), a006195. https://doi.org/10.1101/mcs.a006195

Publication Date

2022-06-23

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Pathology and Cell Biology; Division of Clinical Genetics; Department of Pediatrics

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Murari, A., Goparaju, N. S. V., Rhooms, S.-K., Hossain, K. F. B., Liang, F. G., Garcia, C. J., Osei, C., Liu, T., Li, H., Kitsis, R. N., Patel, R., & Owusu-Ansah, E. (2022). IDH2-mediated regulation of the biogenesis of the oxidative phosphorylation system. Science Advances, 8(19). https://doi.org/10.1126/sciadv.abl8716

Publication Date

2022-05-11

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Physiology and Cellular Biophysics; Robert N. Butler Columbia Aging Center

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Chang, Y.-H., Kang, E. Y.-C., Liu, P.-K., Levi, S. R., Wang, H.-H., Tseng, Y.-J., Seo, G. H., Lee, H., Yeh, L.-K., Chen, K.-J., Wu, W.-C., Lai, C.-C., Liu, L., & Wang, N.-K. (2022). Photoreceptor Manifestations of Primary Mitochondrial Optic Nerve Disorders. Investigative Opthalmology & Visual Science, 63(5), 5. https://doi.org/10.1167/iovs.63.5.5

Publication Date

2022-05-04

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Ophthalmology

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Faitg, J., Lacefield, C., Davey, T., White, K., Laws, R., Kosmidis, S., Reeve, A. K., Kandel, E. R., Vincent, A. E., & Picard, M. (2021). 3D neuronal mitochondrial morphology in axons, dendrites, and somata of the aging mouse hippocampus. Cell Reports, 36(6), 109509. https://doi.org/10.1016/j.celrep.2021.109509

Publication Date

2021-08-12

Columbia Affiliation

Vagelos College of Physicians and Surgeons; Department of Psychiatry; Division of Behavioral Medicine; Zuckerman Mind, Brain, and Behavior Institute; Kavli Institute for Brain Science; Department of Neuroscience; Department of Neurology; H. Houston Merritt Center for Neuromuscular and Mitochondrial Disorders

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