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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 106187964.

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 101487075.

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 93605346.

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 89006977.

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 84892568.

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 82919719.

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 739771410.

Serine catabolism feeds NADH when respiration is impaired

Lifeng Yang, Juan Carlos Garcia Canaveras, Zihong Chen, Lin Wang, Lingfan Liang, Cholsoon Jang, Johannes A. Mayr, Zhaoyue Zhang, Jonathan M. Ghergurovich, Le Zhan, Shilpy Joshi, Zhixian Hu, Melanie R. McReynolds, Xiaoyang Su, Eileen White, Raphael J. Morscher, Joshua D. Rabinowitz

Cell Metab. Author manuscript; available in PMC 2021 Apr 7.

Published in final edited form as: Cell Metab. 2020 Apr 7; 31(4): 809–821.e6. Published online 2020 Mar 17. doi: 10.1016/j.cmet.2020.02.017

PMCID:: PMC7397714

Abstract Article PDF–2.5M

Select item 727214111.

Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease

Simon C. Johnson, Ernst-Bernhard Kayser, Rebecca Bornstein, Julia Stokes, Alessandro Bitto, Kyung Yeon Park, Amanda Pan, Grace Sun, Daniel Raftery, Matt Kaeberlein, Margaret M Sedensky, Philip G Morgan

Mol Genet Metab. Author manuscript; available in PMC 2021 Jun 1.

Published in final edited form as: Mol Genet Metab. 2020 Jun; 130(2): 118–132. Published online 2020 Apr 3. doi: 10.1016/j.ymgme.2020.03.007

PMCID:: PMC7272141

Abstract Article PDF–3.0M

Select item 726682012.

Impaired mitochondrial complex I function as a candidate driver in the biological stress response and a concomitant stress-induced brain metabolic reprogramming in male mice

Tim L. Emmerzaal, Graeme Preston, Bram Geenen, Vivienne Verweij, Maximilian Wiesmann, Elisavet Vasileiou, Femke Grüter, Corné de Groot, Jeroen Schoorl, Renske de Veer, Monica Roelofs, Martijn Arts, Yara Hendriksen, Eva Klimars, Taraka R. Donti, Brett H. Graham, Eva Morava, Richard J. Rodenburg, Tamas Kozicz

Transl Psychiatry. 2020; 10: 176. Published online 2020 Jun 1. doi: 10.1038/s41398-020-0858-y

PMCID:: PMC7266820

Abstract Article PDF–2.0M

Select item 726424513.

Defective NADPH production in mitochondrial disease complex I causes inflammation and cell death

Eduardo Balsa, Elizabeth A. Perry, Christopher F. Bennett, Mark Jedrychowski, Steven P. Gygi, John G. Doench, Pere Puigserver

Nat Commun. 2020; 11: 2714. Published online 2020 Jun 1. doi: 10.1038/s41467-020-16423-1

PMCID:: PMC7264245

Abstract Article PDF–1.2M

Select item 669983814.

Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling

Sidney M. Gospe, III, Amanda M. Travis, Alexander V. Kolesnikov, Mikael Klingeborn, Luyu Wang, Vladimir J. Kefalov, Vadim Y. Arshavsky

J Biol Chem. 2019 Aug 16; 294(33): 12432–12443. Published online 2019 Jun 27. doi: 10.1074/jbc.RA119.007945

PMCID:: PMC6699838

Abstract Article PDF–3.3M

Select item 669347015.

Mitochondrial respiratory chain deficiency inhibits lysosomal hydrolysis

Lorena Fernandez-Mosquera, King Faisal Yambire, Renata Couto, Leonardo Pereyra, Kamil Pabis, Amy H. Ponsford, Cátia V. Diogo, Massimiliano Stagi, Ira Milosevic, Nuno Raimundo

Autophagy. 2019; 15(9): 1572–1591. Published online 2019 Mar 27. doi: 10.1080/15548627.2019.1586256

PMCID:: PMC6693470

Abstract Article PDF–7.5M

Select item 639734516.

Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome

Grigorij Schleifer, Eizo Marutani, Michele Ferrari, Rohit Sharma, Owen Skinner, Olga Goldberger, Robert Matthew Henry Grange, Kathryn Peneyra, Rajeev Malhotra, Martin Wepler, Fumito Ichinose, Donald B. Bloch, Vamsi K. Mootha, Warren M. Zapol

Am J Physiol Lung Cell Mol Physiol. 2019 Feb 1; 316(2): L391–L399. Published online 2018 Dec 6. doi: 10.1152/ajplung.00419.2018

PMCID:: PMC6397345

Abstract Article

Select item 568560817.

Regional knockdown of NDUFS4 implicates a thalamocortical circuit mediating anesthetic sensitivity

Renjini Ramadasan-Nair, Jessica Hui, Pavel I. Zimin, Leslie S. Itsara, Philip G. Morgan, Margaret M. Sedensky

PLoS One. 2017; 12(11): e0188087. Published online 2017 Nov 14. doi: 10.1371/journal.pone.0188087

PMCID:: PMC5685608

Abstract Article PDF–12M

Select item 544816718.

Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome

Michele Ferrari, Isha H. Jain, Olga Goldberger, Emanuele Rezoagli, Robrecht Thoonen, Kai-Hung Cheng, David E. Sosnovik, Marielle Scherrer-Crosbie, Vamsi K. Mootha, Warren M. Zapol

Proc Natl Acad Sci U S A. 2017 May 23; 114(21): E4241–E4250. Published online 2017 May 8. doi: 10.1073/pnas.1621511114

Correction in:: Proc Natl Acad Sci U S A. 2017 Jun 13; 114(24): E4894–E4896.

Correction in:: Proc Natl Acad Sci U S A. 2018 Feb 6; 115(6): E1330.

PMCID:: PMC5448167

Abstract Article PDF–2.0M

Select item 473966619.

Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome

Gerardo G. Piroli, Allison M. Manuel, Anna C. Clapper, Michael D. Walla, John E. Baatz, Richard D. Palmiter, Albert Quintana, Norma Frizzell

Mol Cell Proteomics. 2016 Feb; 15(2): 445–461. Published online 2015 Oct 8. doi: 10.1074/mcp.M115.051516

PMCID:: PMC4739666

Abstract Article PDF–2.5M

Select item 466692720.

Mitochondrial flash as a novel biomarker of mitochondrial respiration in the heart

Guohua Gong, Xiaoyun Liu, Huiliang Zhang, Shey-Shing Sheu, Wang Wang

Am J Physiol Heart Circ Physiol. 2015 Oct; 309(7): H1166–H1177. Published online 2015 Aug 14. doi: 10.1152/ajpheart.00462.2015

PMCID:: PMC4666927

Abstract Article

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