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Select item 110849371.

The Blood–Brain Barrier Is Unaffected in the Ndufs4^−/− Mouse Model of Leigh Syndrome

Robin Reynaud-Dulaurier, Romain Clément, Sara Yjjou, Cassandra Cresson, Yasmina Saoudi, Mathilde Faideau, Michael Decressac

Int J Mol Sci. 2024 May; 25(9): 4828. Published online 2024 Apr 29. doi: 10.3390/ijms25094828

PMCID:: PMC11084937

Abstract Article PDF–1.8M

Select item 109121982.

NDUFS4 regulates cristae remodeling in diabetic kidney disease

Koki Mise, Jianyin Long, Daniel L. Galvan, Zengchun Ye, Guizhen Fan, Rajesh Sharma, Irina I. Serysheva, Travis I. Moore, Collene R. Jeter, M. Anna Zal, Motoo Araki, Jun Wada, Paul T. Schumacker, Benny H. Chang, Farhad R. Danesh

Nat Commun. 2024; 15: 1965. Published online 2024 Mar 4. doi: 10.1038/s41467-024-46366-w

Correction in:: Nat Commun. 2024; 15: 3022.

PMCID:: PMC10912198

Abstract Article PDF–6.2M

Select item 108974353.

Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4^−/− mouse

Zhan Yin, Ahmed-Noor A Agip, Hannah R Bridges, Judy Hirst

EMBO J. 2024 Jan; 43(2): 225–249. Published online 2024 Jan 2. doi: 10.1038/s44318-023-00001-4

PMCID:: PMC10897435

Abstract Article PDF–12M

Select item 106216164.

Elevated susceptibility to exogenous seizure triggers and impaired interneuron excitability in a mouse model of Leigh syndrome epilepsy

Arena Manning, Victor Han, Alexa Stephens, Rose Wang, Nicholas Bush, Michelle Bard, Jan M. Ramirez, Franck Kalume

Neurobiol Dis. Author manuscript; available in PMC 2023 Nov 2.

Published in final edited form as: Neurobiol Dis. 2023 Oct 15; 187: 106288. Published online 2023 Sep 11. doi: 10.1016/j.nbd.2023.106288

PMCID:: PMC10621616

Abstract Article PDF–2.1M

Select item 106187965.

Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency

Gerardo G. Piroli, Allison M. Manuel, Richard S. McCain, Holland H. Smith, Oliver Ozohanics, Sara Mellid, J. Hunter Cox, William E. Cotham, Michael D. Walla, Alberto Cascón, Attila Ambrus, Norma Frizzell

Redox Biol. 2023 Nov; 67: 102932. Published online 2023 Oct 17. doi: 10.1016/j.redox.2023.102932

PMCID:: PMC10618796

Abstract Article PDF–8.3M

Select item 105800156.

Peripheral macrophages drive CNS disease in the Ndufs4(−/−) model of Leigh syndrome

Allison R. Hanaford, Asheema Khanna, Vivian Truong, Katerina James, Yihan Chen, Michael Mulholland, Bernhard Kayser, Ryan W. Liao, Margaret Sedensky, Phil Morgan, Nathan Andrew Baertsch, Vandana Kalia, Surojit Sarkar, Simon C. Johnson

Brain Pathol. 2023 Nov; 33(6): e13192. Published online 2023 Aug 8. doi: 10.1111/bpa.13192

PMCID:: PMC10580015

Abstract Article PDF–4.2M

Select item 103078527.

KCTD13-mediated ubiquitination and degradation of GluN1 regulates excitatory synaptic transmission and seizure susceptibility

Juan Gu, Pingyang Ke, Haokun Guo, Jing Liu, Yan Liu, Xin Tian, Zhuo Huang, Xin Xu, Demei Xu, Yuanlin Ma, Xuefeng Wang, Fei Xiao

Cell Death Differ. 2023 Jul; 30(7): 1726–1741. Published online 2023 May 4. doi: 10.1038/s41418-023-01174-5

PMCID:: PMC10307852

Abstract Article PDF–8.8M

Select item 102474548.

TREK-1 and TREK-2 knockout mice are not resistant to halothane or isoflurane

Kira A Spencer, Christian B Woods, Hailey M Worstman, Simon C Johnson, Jan-Marino Ramirez, Philip G Morgan, Margaret M Sedensky

Anesthesiology. Author manuscript; available in PMC 2024 Jul 1.

Published in final edited form as: Anesthesiology. 2023 Jul 1; 139(1): 63–76. doi: 10.1097/ALN.0000000000004577

PMCID:: PMC10247454

Abstract Article PDF–1.3M

Select item 101487079.

Oxygen Toxicity Causes Cyclic Damage by Destabilizing Specific Fe-S Cluster-Containing Protein Complexes

Alan H. Baik, Augustinus G. Haribowo, Xuewen Chen, Bruno B. Queliconi, Alec M. Barrios, Ankur Garg, Mazharul Maishan, Alexandre R. Campos, Michael A. Matthay, Isha H. Jain

Mol Cell. Author manuscript; available in PMC 2024 Mar 16.

Published in final edited form as: Mol Cell. 2023 Mar 16; 83(6): 942–960.e9. Published online 2023 Mar 8. doi: 10.1016/j.molcel.2023.02.013

PMCID:: PMC10148707

Abstract Article PDF–3.2M

Select item 993821610.

Rescue of neuropsychiatric phenotypes in a mouse model of 16p11.2 duplication syndrome by genetic correction of an epilepsy network hub

Marc P. Forrest, Marc Dos Santos, Nicolas H. Piguel, Yi-Zhi Wang, Nicole A. Hawkins, Vikram A. Bagchi, Leonardo E. Dionisio, Sehyoun Yoon, Dina Simkin, Maria Dolores Martin-de-Saavedra, Ruoqi Gao, Katherine E. Horan, Alfred L. George, Jr., Mark S. LeDoux, Jennifer A. Kearney, Jeffrey N. Savas, Peter Penzes

Nat Commun. 2023; 14: 825. Published online 2023 Feb 17. doi: 10.1038/s41467-023-36087-x

PMCID:: PMC9938216

Abstract Article PDF–5.3M

Select item 954468611.

Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome

Kevin Aguilar, Gemma Comes, Carla Canal, Albert Quintana, Elisenda Sanz, Juan Hidalgo

Glia. 2022 Nov; 70(11): 2032–2044. Published online 2022 Jun 30. doi: 10.1002/glia.24234

PMCID:: PMC9544686

Abstract Article PDF–8.3M

Select item 936053412.

Noninvasive Ophthalmic Imaging Measures Retinal Degeneration and Vision Deficits in Ndufs4^−/− Mouse Model of Mitochondrial Complex I Deficiency

Maria I. Avrutsky, Jacqueline M. Lawson, Jade E. Smart, Claire W. Chen, Carol M. Troy

Transl Vis Sci Technol. 2022 Aug; 11(8): 5. Published online 2022 Aug 3. doi: 10.1167/tvst.11.8.5

PMCID:: PMC9360534

Abstract Article PDF–8.2M

Select item 932920413.

Isoflurane inhibition of endocytosis is an anesthetic mechanism of action

Sangwook Jung, Pavel I Zimin, Christian B Woods, Ernst-Bernhard Kayser, Dominik Haddad, Colleen R Reczek, Ken Nakamura, Jan-Marino Ramirez, Margaret M Sedensky, Philip G Morgan

Curr Biol. Author manuscript; available in PMC 2023 Jul 25.

Published in final edited form as: Curr Biol. 2022 Jul 25; 32(14): 3016–3032.e3. Published online 2022 Jun 9. doi: 10.1016/j.cub.2022.05.037

PMCID:: PMC9329204

Abstract Article PDF–2.8M

Select item 930954114.

Metabolic rescue ameliorates mitochondrial encephalo‐cardiomyopathy in murine and human iPSC models of Leigh syndrome

Jin‐Young Yoon, Nastaran Daneshgar, Yi Chu, Biyi Chen, Marco Hefti, Ajit Vikram, Kaikobad Irani, Long‐Sheng Song, Charles Brenner, E. Dale Abel, Barry London, Dao‐Fu Dai

Clin Transl Med. 2022 Jul; 12(7): e954. Published online 2022 Jul 25. doi: 10.1002/ctm2.954

PMCID:: PMC9309541

Abstract Article PDF–5.7M

Select item 901719215.

Comparative analysis of CI- and CIV-containing respiratory supercomplexes at single-cell resolution

Fabio Bertan, Lena Wischhof, Enzo Scifo, Mihaela Guranda, Joshua Jackson, Anaïs Marsal-Cots, Antonia Piazzesi, Miriam Stork, Michael Peitz, Jochen Herbert Martin Prehn, Dan Ehninger, Pierluigi Nicotera, Daniele Bano

Cell Rep Methods. 2021 May 24; 1(1): 100002. Published online 2021 Apr 2. doi: 10.1016/j.crmeth.2021.100002

PMCID:: PMC9017192

Abstract Article PDF–5.9M

Select item 896710716.

Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention

Melissa A E van de Wal, Merel J W Adjobo-Hermans, Jaap Keijer, Tom J J Schirris, Judith R Homberg, Mariusz R Wieckowski, Sander Grefte, Evert M van Schothorst, Clara van Karnebeek, Albert Quintana, Werner J H Koopman

Brain. 2022 Jan; 145(1): 45–63. Published online 2021 Nov 29. doi: 10.1093/brain/awab426

PMCID:: PMC8967107

Abstract Article PDF–1.8M

Select item 890069717.

Adipose Mitochondrial Complex I Deficiency Modulates Inflammation and Glucose Homeostasis in a Sex-Dependent Manner

Kyung-Mi Choi, Karen K Ryan, John C Yoon

Endocrinology. 2022 Apr; 163(4): bqac018. Published online 2022 Feb 16. doi: 10.1210/endocr/bqac018

PMCID:: PMC8900697

Abstract Article

Select item 848925618.

Hypoxia ameliorates brain hyperoxia and NAD⁺ deficiency in a murine model of Leigh syndrome

Robert M. H. Grange, Rohit Sharma, Hardik Shah, Bryn Reinstadler, Olga Goldberger, Marissa K. Cooper, Akito Nakagawa, Yusuke Miyazaki, Allyson G. Hindle, Annabelle J. Batten, Gregory R. Wojtkiewicz, Grigorij Schleifer, Aranya Bagchi, Eizo Marutani, Rajeev Malhotra, Donald B. Bloch, Fumito Ichinose, Vamsi K. Mootha, Warren M. Zapol

Mol Genet Metab. Author manuscript; available in PMC 2021 Oct 4.

Published in final edited form as: Mol Genet Metab. 2021 May; 133(1): 83–93. Published online 2021 Mar 11. doi: 10.1016/j.ymgme.2021.03.005

PMCID:: PMC8489256

Abstract Article PDF–764K

Select item 829197119.

Mechanisms underlying neonate-specific metabolic effects of volatile anesthetics

Julia Stokes, Arielle Freed, Rebecca Bornstein, Kevin N Su, John Snell, Amanda Pan, Grace X Sun, Kyung Yeon Park, Sangwook Jung, Hailey Worstman, Brittany M Johnson, Philip G Morgan, Margaret M Sedensky, Simon C Johnson

eLife. 2021; 10: e65400. Published online 2021 Jul 13. doi: 10.7554/eLife.65400

PMCID:: PMC8291971

Abstract Article PDF–4.2M

Select item 815511620.

Ndufs4 ablation decreases synaptophysin expression in hippocampus

Subrata Kumar Shil, Yoshiteru Kagawa, Banlanjo Abdulaziz Umaru, Fumika Nanto-Hara, Hirofumi Miyazaki, Yui Yamamoto, Shuhei Kobayashi, Chitose Suzuki, Takaaki Abe, Yuji Owada

Sci Rep. 2021; 11: 10969. Published online 2021 May 26. doi: 10.1038/s41598-021-90127-4

PMCID:: PMC8155116

Abstract Article PDF–1.6M

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