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Select item 44169311.

OXPHOS-Mediated Induction of NAD⁺ Promotes Complete Oxidation of Fatty Acids and Interdicts Non-Alcoholic Fatty Liver Disease

Thomas E. Akie, Lijun Liu, Minwoo Nam, Shi Lei, Marcus P. Cooper

PLoS One. 2015; 10(5): e0125617. Published online 2015 May 1. doi: 10.1371/journal.pone.0125617

PMCID:: PMC4416931

Abstract Article PDF–3.2M

Select item 39901602.

Loss of LRPPRC causes ATP synthase deficiency

Arnaud Mourier, Benedetta Ruzzenente, Tobias Brandt, Werner Kühlbrandt, Nils-Göran Larsson

Hum Mol Genet. 2014 May 15; 23(10): 2580–2592. Published online 2014 Jan 6. doi: 10.1093/hmg/ddt652

PMCID:: PMC3990160

Abstract Article PDF–1.3M

Select item 83769673.

Adaptive optimization of the OXPHOS assembly line partially compensates lrpprc-dependent mitochondrial translation defects in mice

Alexanne Cuillerier, Matthieu Ruiz, Caroline Daneault, Anik Forest, Jenna Rossi, Goutham Vasam, George Cairns, Virgilio Cadete, The LSFC Consortium, Christine Des Rosiers, Yan Burelle

Commun Biol. 2021; 4: 989. Published online 2021 Aug 19. doi: 10.1038/s42003-021-02492-5

PMCID:: PMC8376967

Abstract Article PDF–2.5M

Select item 66755474.

Lipidomics unveils lipid dyshomeostasis and low circulating plasmalogens as biomarkers in a monogenic mitochondrial disorder

Matthieu Ruiz, Alexanne Cuillerier, Caroline Daneault, Sonia Deschênes, Isabelle Robillard Frayne, Bertrand Bouchard, Anik Forest, Julie Thompson Legault, The LSFC Consortium, Frederic M. Vaz, John D. Rioux, Yan Burelle, Christine Des Rosiers

JCI Insight. 2019 Jul 25; 4(14): e123231. Published online 2019 Jul 25. doi: 10.1172/jci.insight.123231

PMCID:: PMC6675547

Abstract Article PDF–4.8M

Select item 54732475.

An unexpected role for the transcriptional coactivator isoform NT-PGC-1α in the regulation of mitochondrial respiration in brown adipocytes

Ji Suk Chang, Kyoungsoo Ha

J Biol Chem. 2017 Jun 16; 292(24): 9958–9966. Published online 2017 May 4. doi: 10.1074/jbc.M117.778373

PMCID:: PMC5473247

Abstract Article PDF–1.7M

Select item 45277676.

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation

Marie Lagouge, Arnaud Mourier, Hyun Ju Lee, Henrik Spåhr, Timothy Wai, Christian Kukat, Eduardo Silva Ramos, Elisa Motori, Jakob D. Busch, Stefan Siira, German Mouse Clinic Consortium, Elisabeth Kremmer, Aleksandra Filipovska, Nils-Göran Larsson

PLoS Genet. 2015 Aug; 11(8): e1005423. Published online 2015 Aug 6. doi: 10.1371/journal.pgen.1005423

PMCID:: PMC4527767

Abstract Article PDF–6.8M

Select item 43817297.

Nutrient sensing by the mitochondrial transcription machinery dictates oxidative phosphorylation

Lijun Liu, Minwoo Nam, Wei Fan, Thomas E. Akie, David C. Hoaglin, Guangping Gao, John F. Keaney, Jr., Marcus P. Cooper

J Clin Invest. 2014 Feb 3; 124(2): 768–784. Published online 2014 Jan 16. doi: 10.1172/JCI69413

PMCID:: PMC4381729

Abstract Article PDF–3.3M

Select item 36687128.

The Leucine-rich Pentatricopeptide Repeat-containing Protein (LRPPRC) Does Not Activate Transcription in Mammalian Mitochondria

Julia Harmel, Benedetta Ruzzenente, Mügen Terzioglu, Henrik Spåhr, Maria Falkenberg, Nils-Göran Larsson

J Biol Chem. 2013 May 31; 288(22): 15510–15519. Published online 2013 Apr 18. doi: 10.1074/jbc.M113.471649

PMCID:: PMC3668712

Abstract Article PDF–2.9M

Select item 33088389.

LRP130 Protein Remodels Mitochondria and Stimulates Fatty Acid Oxidation

Lijun Liu, Masato Sanosaka, Shi Lei, Megan L. Bestwick, Joseph H. Frey, Jr., Yulia V. Surovtseva, Gerald S. Shadel, Marcus P. Cooper

J Biol Chem. 2011 Dec 2; 286(48): 41253–41264. Published online 2011 Oct 4. doi: 10.1074/jbc.M111.276121

PMCID:: PMC3308838

Abstract Article PDF–1.9M

Select item 258154110.

Modulation of PGC-1 Coactivator Pathways in Brown Fat Differentiation through LRP130

Marcus P. Cooper, Marc Uldry, Shingo Kajimura, Zoltan Arany, Bruce M. Spiegelman

J Biol Chem. 2008 Nov 14; 283(46): 31960–31967. doi: 10.1074/jbc.M805431200

PMCID:: PMC2581541

Abstract Article PDF–760K

Select item 588608411.

Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion

Alexanne Cuillerier, Shamisa Honarmand, Virgilio J.J. Cadete, Matthieu Ruiz, Anik Forest, Sonia Deschênes, Claudine Beauchamp, LSFC Consortium, Guy Charron, John D. Rioux, Christine Des Rosiers, Eric A. Shoubridge, Yan Burelle

Hum Mol Genet. 2017 Aug 15; 26(16): 3186–3201. Published online 2017 May 31. doi: 10.1093/hmg/ddx202

PMCID:: PMC5886084

Abstract Article PDF–1.1M

Select item 569107412.

LRPPRC-mediated folding of the mitochondrial transcriptome

Stefan J. Siira, Henrik Spåhr, Anne-Marie J. Shearwood, Benedetta Ruzzenente, Nils-Göran Larsson, Oliver Rackham, Aleksandra Filipovska

Nat Commun. 2017; 8: 1532. Published online 2017 Nov 16. doi: 10.1038/s41467-017-01221-z

PMCID:: PMC5691074

Abstract Article PDF–2.0M

Select item 603284513.

A novel histochemistry assay to assess and quantify focal cytochrome c oxidase deficiency

Marie‐Lune Simard, Arnaud Mourier, Laura C Greaves, Robert W Taylor, James B Stewart

J Pathol. 2018 Jul; 245(3): 311–323. Published online 2018 May 14. doi: 10.1002/path.5084

PMCID:: PMC6032845

Abstract Article PDF–11M

Select item 570364414.

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

Inge Kühl, Maria Miranda, Ilian Atanassov, Irina Kuznetsova, Yvonne Hinze, Arnaud Mourier, Aleksandra Filipovska, Nils-Göran Larsson

eLife. 2017; 6: e30952. Published online 2017 Nov 14. doi: 10.7554/eLife.30952

PMCID:: PMC5703644

Abstract Article PDF–6.0M

Select item 326155715.

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

Benedetta Ruzzenente, Metodi D Metodiev, Anna Wredenberg, Ana Bratic, Chan Bae Park, Yolanda Cámara, Dusanka Milenkovic, Volker Zickermann, Rolf Wibom, Kjell Hultenby, Hediye Erdjument-Bromage, Paul Tempst, Ulrich Brandt, James B Stewart, Claes M Gustafsson, Nils-Göran Larsson

EMBO J. 2012 Jan 18; 31(2): 443–456. Published online 2011 Nov 1. doi: 10.1038/emboj.2011.392

PMCID:: PMC3261557

Abstract Article PDF–1.1M

Select item 920252316.

Brown adipocyte ATF4 activation improves thermoregulation and systemic metabolism

Esther Paulo, Yun Zhang, Ruchi Masand, Tony L. Huynh, Youngho Seo, Danielle L. Swaney, Margaret Soucheray, Erica Stevenson, David Jimenez-Morales, Nevan J. Krogan, Biao Wang

Cell Rep. Author manuscript; available in PMC 2022 Jun 16.

Published in final edited form as: Cell Rep. 2021 Sep 21; 36(12): 109742. doi: 10.1016/j.celrep.2021.109742