Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

Transcriptional silencing and longevity protein Sir2 is an NAD-dependent histone deacetylase

Imai, S.-I., Armstrong, C. M., Kaeberlein, M., & Guarente, L. (2000) · Nature · doi:10.1038/35001622 · Cited by 3,510 · see citing works

Mitochondria: In Sickness and in Health

Nunnari, J., & Suomalainen, A. (2012) · Cell · doi:10.1016/j.cell.2012.02.035 · Cited by 3,479 · see citing works

AMPK regulates energy expenditure by modulating NAD+ metabolism and SIRT1 activity

Cantó, C., Gerhart-Hines, Z., Feige, J. N., Lagouge, M., Noriega, L., Milne, J. C., Elliott, P. J., Puigserver, P., & Auwerx, J. (2009) · Nature · doi:10.1038/nature07813 · Cited by 3,300 · see citing works

Stress-dependent regulation of FOXO transcription factors by the SIRT1 deacetylase

Brunet, A., Sweeney, L. B., Sturgill, J. F., Chua, K. F., Greer, P. L., Lin, Y., Tran, H., Ross, S. E., Mostoslavsky, R., Cohen, H. Y., et al. (2004) · Science · doi:10.1126/science.1094637 · Cited by 3,239 · see citing works

Nutrient control of glucose homeostasis through a complex of PGC-1α and SIRT1

Rodgers, J. T., Lerin, C., Haas, W., Gygi, S. P., Spiegelman, B. M., & Puigserver, P. (2005) · Nature · doi:10.1038/nature03354 · Cited by 3,160 · see citing works

FGF-21 as a novel metabolic regulator

Kharitonenkov, A., Shiyanova, T. L., Koester, A., Ford, A. M., Micanovic, R., Galbreath, E. J., Sandusky, G. E., Hammond, L. J., Moyers, J. S., Owens, R. A., et al. (2005) · Journal of Clinical Investigation · doi:10.1172/jci23606 · Cited by 2,125 · see citing works

Declining NAD+ Induces a Pseudohypoxic State Disrupting Nuclear-Mitochondrial Communication during Aging

Gomes, A. P., Price, N. L., Ling, A. J. Y., Moslehi, J. J., Montgomery, M. K., Rajman, L., White, J. P., Teodoro, J. S., Wrann, C. D., Hubbard, B. P., et al. (2013) · Cell · doi:10.1016/j.cell.2013.11.037 · Cited by 1,482 · see citing works

The NAD+ Precursor Nicotinamide Riboside Enhances Oxidative Metabolism and Protects against High-Fat Diet-Induced Obesity

Cantó, C., Houtkooper, R. H., Pirinen, E., Youn, D. Y., Oosterveer, M. H., Cen, Y., Fernandez-Marcos, P. J., Yamamoto, H., Andreux, P. A., Cettour-Rose, P., et al. (2012) · Cell Metabolism · doi:10.1016/j.cmet.2012.04.022 · Cited by 1,238 · see citing works

The NAD+/Sirtuin Pathway Modulates Longevity through Activation of Mitochondrial UPR and FOXO Signaling

Mouchiroud, L., Houtkooper, R. H., Moullan, N., Katsyuba, E., Ryu, D., Cantó, C., Mottis, A., Jo, Y.-S., Viswanathan, M., Schoonjans, K., et al. (2013) · Cell · doi:10.1016/j.cell.2013.06.016 · Cited by 1,210 · see citing works

The Cell Non-Autonomous Nature of Electron Transport Chain-Mediated Longevity

Durieux, J., Wolff, S., & Dillin, A. (2011) · Cell · doi:10.1016/j.cell.2010.12.016 · Cited by 1,059 · see citing works

Mitonuclear protein imbalance as a conserved longevity mechanism

Houtkooper, R. H., Mouchiroud, L., Ryu, D., Moullan, N., Katsyuba, E., Knott, G., Williams, R. W., & Auwerx, J. (2013) · Nature · doi:10.1038/nature12188 · Cited by 1,035 · see citing works

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

Nargund, A. M., Pellegrino, M., Fiorese, C. J., Baker, B. M., & Haynes, C. M. (2012) · Science · doi:10.1126/science.1223560 · Cited by 1,027 · see citing works

The secret life of NAD+: an old metabolite controlling new metabolic signaling pathways

Houtkooper, R. H., Cantó, C., Wanders, R. J., & Auwerx, J. (2010) · Endocrine Reviews · doi:10.1210/er.2009-0026 · Cited by 899 · see citing works

Discoveries of Nicotinamide Riboside as a Nutrient and Conserved NRK Genes Establish a Preiss-Handler Independent Route to NAD+ in Fungi and Humans

Bieganowski, P., & Brenner, C. (2004) · Cell · doi:10.1016/s0092-8674(04)00416-7 · Cited by 750 · see citing works

[42]Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines

Trounce, I. A., Kim, Y. L., Jun, A. S., & Wallace, D. C. (1996) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(96)64044-0 · Cited by 749 · see citing works

An autosomal dominant disorder with multiple deletions of mitochondrial DNA starting at the D-loop region

Zeviani, M., Servidei, S., Gellera, C., Bertini, E., DiMauro, S., & DiDonato, S. (1989) · Nature · doi:10.1038/339309a0 · Cited by 636 · see citing works

ClpP Mediates Activation of a Mitochondrial Unfolded Protein Response in C. elegans

Haynes, C. M., Petrova, K., Benedetti, C., Yang, Y., & Ron, D. (2007) · Developmental Cell · doi:10.1016/j.devcel.2007.07.016 · Cited by 595 · see citing works

Bioenergetic Analysis of Peroxisome Proliferator-activated Receptor γ Coactivators 1α and 1β (PGC-1α and PGC-1β) in Muscle Cells

St‐Pierre, J., Lin, J. D., Krauss, S., Tarr, P. T., Yang, R., Newgard, C. B., & Spiegelman, B. M. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m301850200 · Cited by 543 · see citing works

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

Johnson, S. C., Yanos, M. E., Kayser, E., Quintana, A., Sangesland, M., Castanza, A. S., Uhde, L., Hui, J., Wall, V., Gagnidze, A., et al. (2013) · Science · doi:10.1126/science.1244360 · Cited by 520 · see citing works

FGF-21 as a biomarker for muscle-manifesting mitochondrial respiratory chain deficiencies: a diagnostic study

Suomalainen, A., Elo, J. M., Pietiläinen, K. H., Hakonen, A. H., Sevastianova, K., Korpela, M., Isohanni, P., Marjavaara, S. K., Tyni, T., Kiuru-Enari, S., et al. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70155-7 · Cited by 427 · see citing works

Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice

Tyynismaa, H., Mjosund, K. P., Wanrooij, S., Lappalainen, I., Ylikallio, E., Jalanko, A., Spelbrink, J. N., Paetau, A., & Suomalainen, A. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0505551102 · Cited by 342 · see citing works

Mitochondrial myopathy induces a starvation-like response

Tyynismaa, H., Carroll, C. J., Raimundo, N., Ahola-Erkkilä, S., Wenz, T., Ruhanen, H., Guse, K., Hemminki, A., Peltola-Mjøsund, K. E., Tulkki, V., et al. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq310 · Cited by 300 · see citing works

In Vivo Correction of COX Deficiency by Activation of the AMPK/PGC-1α Axis

Viscomi, C. F., Bottani, E., Civiletto, G., Cerutti, R., Moggio, M. G., Fagiolari, G., Schon, E. A., Lamperti, C., & Zeviani, M. (2011) · Cell Metabolism · doi:10.1016/j.cmet.2011.04.011 · Cited by 291 · see citing works

Multiple deletions of mitochondrial DNA in several tissues of a patient with severe retarded depression and familial progressive external ophthalmoplegia.

Suomalainen, A., Majander, A. S., Haltia, M., Somer, H., Lönnqvist, J. K., Savontaus, M. L., & Peltonen, L. (1992) · Journal of Clinical Investigation · doi:10.1172/jci115856 · Cited by 240 · see citing works

Evaluating and responding to mitochondrial dysfunction: the mitochondrial unfolded-protein response and beyond

Haynes, C. M., Fiorese, C. J., & Lin, Y. (2013) · Trends in Cell Biology · doi:10.1016/j.tcb.2013.02.002 · Cited by 209 · see citing works

Ketogenic diet slows down mitochondrial myopathy progression in mice

Ahola, S., Carroll, C. J., Mjøsund, K., Tulkki, V., Mattila, I. M., Seppänen‐Laakso, T., Orešič, M., Tyynismaa, H., & Suomalainen, A. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq076 · Cited by 191 · see citing works

Mechanisms of mitochondrial diseases

Ylikallio, E., & Suomalainen, A. (2011) · Annals of Medicine · doi:10.3109/07853890.2011.598547 · Cited by 185 · see citing works

Autosomal dominant progressive external ophthalmoplegia with multiple deletions of mtDNA

Suomalainen, A., Majander, A. S., Wallin, M., Setälä, K., KONTULA, K. K., Leinonen, H., Salmi, T. T., Paetau, A. E., Haltia, M., Valanne, L. K., et al. (1997) · Neurology · doi:10.1212/wnl.48.5.1244 · Cited by 140 · see citing works

Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice

Yatsuga, S., & Suomalainen, A. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr482 · Cited by 124 · see citing works

Syntheses of Nicotinamide Riboside and Derivatives: Effective Agents for Increasing Nicotinamide Adenine Dinucleotide Concentrations in Mammalian Cells

Yang, T., Chan, N. Y., & Sauve, A. A. (2007) · Journal of Medicinal Chemistry · doi:10.1021/jm701001c · Cited by 113 · see citing works

Multiple Symmetrical Lipomatosis — A mitochondrial disorder of brown fat

Plummer, C., Spring, P. J., Marotta, R., Chin, J., Taylor, G., Sharpe, D., Athanasou, N. A., Thyagarajan, D. E., & Berkovic, S. F. (2013) · Mitochondrion · doi:10.1016/j.mito.2013.03.003 · Cited by 80 · see citing works

Therapy for mitochondrial disorders: Little proof, high research activity, some promise

Suomalainen, A. (2011) · Seminars in Fetal and Neonatal Medicine · doi:10.1016/j.siny.2011.05.003 · Cited by 47 · see citing works

Brown adipose tissue functions in humans

Virtanen, K. A., Lichtenbelt, W. D. V. M., & Nuutila, P. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2012.12.011 · Cited by 31 · see citing works

Mitophagy and Quality Control Mechanisms in Mitochondrial Maintenance

Pickles, S., Vigié, P., & Youle, R. J. (2018) · Current Biology · doi:10.1016/j.cub.2018.01.004 · Cited by 2,081 · see citing works

Sarcopenia: Aging-Related Loss of Muscle Mass and Function

Larsson, L., Degens, H., Li, M., Salviati, L., Lee, Y. I., Thompson, W., Kirkland, J. L., & Sandri, M. (2019) · Physiological Reviews · doi:10.1152/physrev.00061.2017 · Cited by 1,797 · see citing works

NAD+ Metabolism and the Control of Energy Homeostasis: A Balancing Act between Mitochondria and the Nucleus

Cantó, C., Menzies, K. J., & Auwerx, J. (2015) · Cell Metabolism · doi:10.1016/j.cmet.2015.05.023 · Cited by 1,648 · see citing works

Mitochondrial diseases

Gorman, G. S., Chinnery, P. F., DiMauro, S., Hirano, M., Koga, Y., McFarland, R., Suomalainen, A., Thorburn, D. R., Zeviani, M., & Turnbull, D. M. (2016) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2016.80 · Cited by 1,615 · see citing works

NAD ⁺ in aging, metabolism, and neurodegeneration

Verdin, E. (2015) · Science · doi:10.1126/science.aac4854 · Cited by 1,319 · see citing works

NAD+ Intermediates: The Biology and Therapeutic Potential of NMN and NR

Yoshino, J., Baur, J. A., & Imai, S.-I. (2018) · Cell Metabolism · doi:10.1016/j.cmet.2017.11.002 · Cited by 1,061 · see citing works

Long-Term Administration of Nicotinamide Mononucleotide Mitigates Age-Associated Physiological Decline in Mice

Mills, K. F., Yoshida, S., Stein, L. R., Grozio, A., Kubota, S., Sasaki, Y., Redpath, P., Migaud, M. E., Apte, R. S., Uchida, K., et al. (2016) · Cell Metabolism · doi:10.1016/j.cmet.2016.09.013 · Cited by 930 · see citing works

Metabolic control of autophagy

Galluzzi, L., Pietrocola, F., Levine, B., & Kroemer, G. (2014) · Cell · doi:10.1016/j.cell.2014.11.006 · Cited by 855 · see citing works

Slowing ageing by design: the rise of NAD+ and sirtuin-activating compounds

Bonkowski, M. S., & Sinclair, D. A. (2016) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2016.93 · Cited by 834 · see citing works

Mitonuclear communication in homeostasis and stress

Quirós, P. M., Mottis, A., & Auwerx, J. (2016) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2016.23 · Cited by 787 · see citing works

The mitochondrial UPR: mechanisms, physiological functions and implications in ageing

Shpilka, T., & Haynes, C. M. (2017) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2017.110 · Cited by 716 · see citing works

NAD+ homeostasis in health and disease

Katsyuba, E., Romani, M., Hofer, D., & Auwerx, J. (2020) · Nature Metabolism · doi:10.1038/s42255-019-0161-5 · Cited by 627 · see citing works

Mitochondrial diseases: the contribution of organelle stress responses to pathology

Suomalainen, A., & Battersby, B. J. (2018) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2017.66 · Cited by 505 · see citing works

NAD + metabolism: Bioenergetics, signaling and manipulation for therapy

Yang, Y. J., & Sauve, A. A. (2016) · Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics · doi:10.1016/j.bbapap.2016.06.014 · Cited by 488 · see citing works

Leigh syndrome: One disorder, more than 75 monogenic causes

Lake, N. J., Compton, A. G., Rahman, S., & Thorburn, D. R. (2015) · Annals of Neurology · doi:10.1002/ana.24551 · Cited by 499 · see citing works

AMPK in skeletal muscle function and metabolism

Kjøbsted, R., Hingst, J. R., Fentz, J., Foretz, M., Sanz, M.-N., Pehmøller, C. K., Shum, M., Marette, A., Mounier, R., Treebak, J. T., et al. (2017) · The FASEB Journal · doi:10.1096/fj.201700442r · Cited by 496 · see citing works

Mitochondrial Diseases: Hope for the Future

Russell, O. M., Gorman, G. S., Lightowlers, R. N., & Turnbull, D. M. (2020) · Cell · doi:10.1016/j.cell.2020.02.051 · Cited by 460 · see citing works

mTORC1 Regulates Mitochondrial Integrated Stress Response and Mitochondrial Myopathy Progression

Khan, N. A., Nikkanen, J., Yatsuga, S., Jackson, C. B., Wang, L., Pradhan, S., Kivelä, R., Pessia, A., Velagapudi, V., & Suomalainen, A. (2017) · Cell Metabolism · doi:10.1016/j.cmet.2017.07.007 · Cited by 391 · see citing works

Eliciting the mitochondrial unfolded protein response by nicotinamide adenine dinucleotide repletion reverses fatty liver disease in mice

Gariani, K., Menzies, K. J., Ryu, D., Wegner, C. J., Wang, X., Ropelle, E. R., Moullan, N., Zhang, H., Perino, A., Lemos, V., et al. (2016) · Hepatology · doi:10.1002/hep.28245 · Cited by 368 · see citing works

Loss of NAD Homeostasis Leads to Progressive and Reversible Degeneration of Skeletal Muscle

Frederick, D. W., Loro, E., Liu, L., Davila, A., Chellappa, K., Silverman, I. M., Quinn, W. J., Gosai, S. J., Tichy, E. D., Davis, J. G., et al. (2016) · Cell Metabolism · doi:10.1016/j.cmet.2016.07.005 · Cited by 367 · see citing works

Nicotinamide Riboside Preserves Cardiac Function in a Mouse Model of Dilated Cardiomyopathy

Diguet, N., Trammell, S. A. J., Tannous, C., Deloux, R., Piquereau, J., Mougenot, N., Gouge, A., Gressette, M., Manoury, B., Blanc, J., et al. (2018) · Circulation · doi:10.1161/circulationaha.116.026099 · Cited by 358 · see citing works

Mitochondrial oxidative capacity and NAD+ biosynthesis are reduced in human sarcopenia across ethnicities

Migliavacca, E., Tay, S. K. H., Patel, H. P., Sonntag, T., Civiletto, G., McFarlane, C., Forrester, T., Barton, S. J., Leow, M. K., Antoun, E., et al. (2019) · Nature Communications · doi:10.1038/s41467-019-13694-1 · Cited by 365 · see citing works

Nicotinamide Improves Aspects of Healthspan, but Not Lifespan, in Mice

Mitchell, S. J., Bernier, M., Aon, M. A., Cortassa, S., Kim, E. Y., Fang, E. F., Palacios, H. H., Ali, A., Navas-Enamorado, I., Di Francesco, A., et al. (2018) · Cell Metabolism · doi:10.1016/j.cmet.2018.02.001 · Cited by 338 · see citing works

Mutations causing mitochondrial disease: What is new and what challenges remain?

Lightowlers, R. N., Taylor, R. W., & Turnbull, D. M. (2015) · Science · doi:10.1126/science.aac7516 · Cited by 341 · see citing works

Repairing mitochondrial dysfunction in disease

Sorrentino, V., Menzies, K. J., & Auwerx, J. (2018) · Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev-pharmtox-010716-104908 · Cited by 337 · see citing works

Protein acetylation in metabolism – metabolites and cofactors

Menzies, K. J., Zhang, H., Katsyuba, E., & Auwerx, J. (2016) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2015.181 · Cited by 331 · see citing works

The pharmacological regulation of cellular mitophagy

Georgakopoulos, N., Wells, G., & Campanella, M. (2017) · Nature Chemical Biology · doi:10.1038/nchembio.2287 · Cited by 318 · see citing works

Evolving concepts in NAD+ metabolism

Chini, C. C. S., Zeidler, J. D., Kashyap, S., Warner, G. M., & Chini, E. N. (2021) · Cell Metabolism · doi:10.1016/j.cmet.2021.04.003 · Cited by 294 · see citing works

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

Nikkanen, J., Forsström, S., Euro, L., Paetau, I., Kohnz, R. A., Wang, L., Chilov, D., Viinamäki, J., Roivainen, A., Marjamäki, P., et al. (2016) · Cell Metabolism · doi:10.1016/j.cmet.2016.01.019 · Cited by 285 · see citing works

Mitochondrial medicine in the omics era

Rahman, J., & Rahman, S. (2018) · The Lancet · doi:10.1016/s0140-6736(18)30727-x · Cited by 287 · see citing works

Kynurenine pathway, NAD+ synthesis, and mitochondrial function: Targeting tryptophan metabolism to promote longevity and healthspan

Castro-Portuguez, R., & Sutphin, G. L. (2020) · Experimental Gerontology · doi:10.1016/j.exger.2020.110841 · Cited by 282 · see citing works

Niacin Cures Systemic NAD+ Deficiency and Improves Muscle Performance in Adult-Onset Mitochondrial Myopathy

Pirinen, E., Auranen, M., Khan, N. A., Brilhante, V., Urho, N., Pessia, A., Hakkarainen, A., Kuula, J., Heinonen, U., Schmidt, M. S., et al. (2020) · Cell Metabolism · doi:10.1016/j.cmet.2020.04.008 · Cited by 279 · see citing works

Lethal Cardiomyopathy in Mice Lacking Transferrin Receptor in the Heart

Xu, W., Barrientos, T., Mao, L., Rockman, H. A., Sauve, A. A., & Andrews, N. C. (2015) · Cell Reports · doi:10.1016/j.celrep.2015.09.023 · Cited by 274 · see citing works

Pleiotropic effects of mitochondria in aging

de Lima, T. I., Li, T. Y., Mottis, A., & Auwerx, J. H. (2022) · Nature Aging · doi:10.1038/s43587-022-00191-2 · Cited by 242 · see citing works

The NAD-booster nicotinamide riboside potently stimulates hematopoiesis through increased mitochondrial clearance

Vannini, N., Campos, V., Girotra, M., Trachsel, V., Rojas-Sutterlin, S., Tratwal, J., Ragusa, S., Stefanidis, E., Ryu, D., Rainer, P. Y., et al. (2019) · Cell Stem Cell · doi:10.1016/j.stem.2019.02.012 · Cited by 218 · see citing works

Modulating NAD+ metabolism, from bench to bedside

Katsyuba, E., & Auwerx, J. H. (2017) · The EMBO Journal · doi:10.15252/embj.201797135 · Cited by 224 · see citing works

Nicotinamide Riboside—The Current State of Research and Therapeutic Uses

Mehmel, M., Jovanović, N. D., & Spitz, U. P. (2020) · Nutrients · doi:10.3390/nu12061616 · Cited by 225 · see citing works

Nicotinamide provides neuroprotection in glaucoma by protecting against mitochondrial and metabolic dysfunction

Tribble, J. R., Otmani, A., Sun, S., Ellis, S. A., Cimaglia, G., Vohra, R., Jöe, M., Lardner, E., Venkataraman, A. P., Domínguez‐Vicent, A., et al. (2021) · Redox Biology · doi:10.1016/j.redox.2021.101988 · Cited by 233 · see citing works

The human NAD metabolome: Functions, metabolism and compartmentalization

Nikiforov, A. A., Kulikova, V., & Ziegler, M. (2015) · Critical Reviews in Biochemistry and Molecular Biology · doi:10.3109/10409238.2015.1028612 · Cited by 214 · see citing works

Emerging therapeutic roles for NAD+ metabolism in mitochondrial and age‐related disorders

Srivastava, S. (2016) · Clinical and Translational Medicine · doi:10.1186/s40169-016-0104-7 · Cited by 193 · see citing works

Mitohormesis, an Antiaging Paradigm

Bárcena, C., Mayoral, P., & Quirós, P. M. (2018) · International review of cytology · doi:10.1016/bs.ircmb.2018.05.002 · Cited by 167 · see citing works

Mitochondrial NAD + /NADH Redox State and Diabetic Cardiomyopathy

Berthiaume, J. M., Kurdys, J., Muntean, D. M., & Rosca, M. G. (2017) · Antioxidants and Redox Signaling · doi:10.1089/ars.2017.7415 · Cited by 168 · see citing works

Nicotinamide riboside supplementation alters body composition and skeletal muscle acetylcarnitine concentrations in healthy obese humans

Remie, C. M., Roumans, K. H., Moonen, M. P., Connell, N. J., Havekes, B., Mevenkamp, J., Lindeboom, L., de Wit, V. H., van de Weijer, T., Aarts, S. A., et al. (2020) · The American Journal of Clinical Nutrition · doi:10.1093/ajcn/nqaa072 · Cited by 160 · see citing works

Acetylation of Mitochondrial Proteins in the Heart: The Role of SIRT3

Parodi‐Rullán, R. M., Chapa‐Dubocq, X. R., & Javadov, S. A. (2018) · Frontiers in Physiology · doi:10.3389/fphys.2018.01094 · Cited by 168 · see citing works

Dihydromyricetin Ameliorates Nonalcoholic Fatty Liver Disease by Improving Mitochondrial Respiratory Capacity and Redox Homeostasis Through Modulation of SIRT3 Signaling

Zeng, X., Yang, J., Hu, O., Huang, J., Li, R., Chen, M., Zhang, Y., Zhou, X., Zhu, J., Zhang, Q., et al. (2018) · Antioxidants and Redox Signaling · doi:10.1089/ars.2017.7172 · Cited by 163 · see citing works

Unravelling the mechanisms regulating muscle mitochondrial biogenesis

Hood, D. A., Tryon, L. D., Carter, H. N., Kim, Y., & Chen, C. C. W. (2016) · Biochemical Journal · doi:10.1042/bcj20160009 · Cited by 159 · see citing works

Emerging therapies for mitochondrial diseases

Hirano, M., Emmanuele, V., & Quinzii, C. M. (2018) · Essays in Biochemistry · doi:10.1042/ebc20170114 · Cited by 161 · see citing works

A model of the mitochondrial basis of bipolar disorder

Morris, G., Walder, K., McGee, S. L., Dean, O. M., Tye, S. J., Maes, M., & Berk, M. (2017) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2017.01.014 · Cited by 162 · see citing works

Metabolic Profiling of Alternative NAD Biosynthetic Routes in Mouse Tissues

Mori, V., Amici, A., Mazzola, F., Stefano, M. D., Conforti, L., Magni, G., Ruggieri, S., Raffaelli, N., & Orsomando, G. (2014) · PLoS ONE · doi:10.1371/journal.pone.0113939 · Cited by 158 · see citing works

Brain SIRT1 Mediates Metabolic Homeostasis and Neuroprotection

Xu, J., Jackson, C. W., Khoury, N., Escobar, I., & Pérez‐Pinzón, M. A. (2018) · Frontiers in Endocrinology · doi:10.3389/fendo.2018.00702 · Cited by 158 · see citing works

Glycerol-3-phosphate biosynthesis regenerates cytosolic NAD+ to alleviate mitochondrial disease

Liu, S., Fu, S., Wang, G., Cao, Y., Li, L., Li, X., Yang, J. J., Li, N., Shan, Y., Cao, Y., et al. (2021) · Cell Metabolism · doi:10.1016/j.cmet.2021.06.013 · Cited by 154 · see citing works

Diagnosis and Treatment of Mitochondrial Myopathies

Ahmed, S. T., Craven, L., Russell, O. M., Turnbull, D. M., & Vincent, A. E. (2018) · Neurotherapeutics · doi:10.1007/s13311-018-00674-4 · Cited by 153 · see citing works

The role of NAD+ metabolism and its modulation of mitochondria in aging and disease

Yusri, K., Jose, S., Vermeulen, K. S., Tan, T. C. M., & Sorrentino, V. (2025) · npj Metabolic Health and Disease · doi:10.1038/s44324-025-00067-0 · Cited by 156 · see citing works

Nicotinamide riboside does not alter mitochondrial respiration, content or morphology in skeletal muscle from obese and insulin‐resistant men

Dollerup, O. L., Chubanava, S., Agerholm, M., Søndergård, S. D., Altıntaş, A., Møller, A. B., Høyer, K. F., Ringgaard, S., Stødkilde‐Jørgensen, H., Lavery, G. G., et al. (2020) · The Journal of Physiology · doi:10.1113/jp278752 · Cited by 145 · see citing works

Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load

Jiang, M., Kauppila, T. E. S., Motori, E., Li, X., Atanassov, I., Folz‐Donahue, K., Bonekamp, N. A., Albarran-Gutierrez, S., Stewart, J. B., & Larsson, N. (2017) · Cell Metabolism · doi:10.1016/j.cmet.2017.07.003 · Cited by 144 · see citing works

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

Balsa, E., Perry, E. A., Bennett, C. F., Jedrychowski, M. P., Gygi, S. P., Doench, J. G., & Puigserver, P. (2020) · Nature Communications · doi:10.1038/s41467-020-16423-1 · Cited by 138 · see citing works

The mitochondrial unfolded protein response–synchronizing genomes

Jovaisaite, V., & Auwerx, J. (2015) · Current Opinion in Cell Biology · doi:10.1016/j.ceb.2014.12.003 · Cited by 131 · see citing works

Cigarette toxicity triggers Leber’s hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways

Giordano, L., Deceglie, S., D’Adamo, P., Valentino, M. L., Morgia, C. L., Fracasso, F., Roberti, M., Cappellari, M., Petrosillo, G., Ciaravolo, S., et al. (2015) · Cell Death and Disease · doi:10.1038/cddis.2015.364 · Cited by 132 · see citing works

Power of mitochondrial drug delivery systems to produce innovative nanomedicines

Yamada, Y., Satrialdi, S., Hibino, M., Sasaki, D., Abe, J., & Harashima, H. (2020) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2020.09.010 · Cited by 129 · see citing works

Mitochondrial DNA: Impacting Central and Peripheral Nervous Systems

Carelli, V., & Chan, D. C. (2014) · Neuron · doi:10.1016/j.neuron.2014.11.022 · Cited by 127 · see citing works

Nicotinamide riboside kinases display redundancy in mediating nicotinamide mononucleotide and nicotinamide riboside metabolism in skeletal muscle cells

Fletcher, R. S., Ratajczak, J., Doig, C. L., Oakey, L. A., Callingham, R., Xavier, G. D. S., Garten, A., Elhassan, Y., Redpath, P., Migaud, M. E., et al. (2017) · Molecular Metabolism · doi:10.1016/j.molmet.2017.05.011 · Cited by 121 · see citing works

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

Lee, C. F., Caudal, A., Abell, L., Gowda, G. A. N., & Tian, R. (2019) · Scientific Reports · doi:10.1038/s41598-019-39419-4 · Cited by 120 · see citing works

Nicotinamide Riboside Is a Major NAD+ Precursor Vitamin in Cow Milk

Trammell, S. A., Yu, L., Redpath, P., Migaud, M. E., & Brenner, C. (2016) · The Journal of Nutrition · doi:10.3945/jn.116.230078 · Cited by 112 · see citing works

Cytosolic Proteostasis Networks of the Mitochondrial Stress Response

D'Amico, D. F., Sorrentino, V., & Auwerx, J. H. (2017) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2017.05.002 · Cited by 106 · see citing works

Lithocholic acid phenocopies anti-ageing effects of calorie restriction

Qu, Q., Chen, Y., Wang, Y., Long, S., Wang, W., Yang, H.-Y., Li, M., Tian, X., Wei, X., Liu, Y., et al. (2024) · Nature · doi:10.1038/s41586-024-08329-5 · Cited by 116 · see citing works

Emerging concepts in the therapy of mitochondrial disease

Viscomi, C. F., Bottani, E., & Zeviani, M. (2015) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/j.bbabio.2015.03.001 · Cited by 107 · see citing works

Mitochondrial function is impaired in the skeletal muscle of pre-frail elderly

Andreux, P. A., van Diemen, M. P. J., Heezen, M. R., Auwerx, J. H., Rinsch, C., Groeneveld, G. J., & Singh, A. (2018) · Scientific Reports · doi:10.1038/s41598-018-26944-x · Cited by 108 · see citing works

Oral Administration of Nicotinamide Mononucleotide Is Safe and Efficiently Increases Blood Nicotinamide Adenine Dinucleotide Levels in Healthy Subjects

Okabe, K., Yaku, K., Uchida, Y., Fukamizu, Y., Sato, T., Sakurai, T., Tobe, K., & Nakagawa, T. (2022) · Frontiers in Nutrition · doi:10.3389/fnut.2022.868640 · Cited by 105 · see citing works

The complex crosstalk between mitochondria and the nucleus: What goes in between?

Cagin, U., & Enrı́quez, J. A. (2015) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2015.01.026 · Cited by 101 · see citing works

NAD+ therapy in age-related degenerative disorders: A benefit/risk analysis

Braidy, N., & Liu, Y. (2020) · Experimental Gerontology · doi:10.1016/j.exger.2020.110831 · Cited by 96 · see citing works

Targeting NAD+ in Metabolic Disease: New Insights Into an Old Molecule

Elhassan, Y., Philp, A., & Lavery, G. G. (2017) · Journal of the Endocrine Society · doi:10.1210/js.2017-00092 · Cited by 92 · see citing works

Mitochondrial disorders in children: toward development of small‐molecule treatment strategies

Koopman, W. J. H., Beyrath, J., Fung, C., Koene, S., Rodenburg, R. J., Willems, P. H. G. M., & Smeitink, J. A. M. (2016) · EMBO Molecular Medicine · doi:10.15252/emmm.201506131 · Cited by 90 · see citing works

Increasing NAD Synthesis in Muscle via Nicotinamide Phosphoribosyltransferase Is Not Sufficient to Promote Oxidative Metabolism

Frederick, D. W., Davis, J. G., Dávila, A., Agarwal, B., Michán, S., Puchowicz, M. A., Nakamaru‐Ogiso, E., & Baur, J. A. (2014) · Journal of Biological Chemistry · doi:10.1074/jbc.m114.579565 · Cited by 88 · see citing works

Cardiolipin-targeted peptides rejuvenate mitochondrial function, remodel mitochondria, and promote tissue regeneration during aging

Szeto, H. H., & Liu, S. (2018) · Archives of Biochemistry and Biophysics · doi:10.1016/j.abb.2018.10.013 · Cited by 87 · see citing works

Upregulation of GPR109A in Parkinson’s Disease

Wakade, C. G., Chong, R. K. Y., Bradley, E., Thomas, B., & Morgan, J. C. (2014) · PLoS ONE · doi:10.1371/journal.pone.0109818 · Cited by 86 · see citing works

Generation, Release, and Uptake of the NAD Precursor Nicotinic Acid Riboside by Human Cells

Kulikova, V., Shabalin, K., Nerinovski, K., Dölle, C., Niere, M., Yakimov, A., Redpath, P., Khodorkovskiy, M., Migaud, M. E., Ziegler, M., et al. (2015) · Journal of Biological Chemistry · doi:10.1074/jbc.m115.664458 · Cited by 84 · see citing works

SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease

Gut, P., Matilainen, S., Meyer, J. G., Pällijeff, P., Richard, J., Carroll, C. J., Euro, L., Jackson, C. B., Isohanni, P., Minassian, B. A., et al. (2020) · Nature Communications · doi:10.1038/s41467-020-19743-4 · Cited by 83 · see citing works

p53 prevents doxorubicin cardiotoxicity independently of its prototypical tumor suppressor activities

Li, J., Wang, P., Long, N., Zhuang, J., Springer, D., Zou, J., Lin, Y., Bleck, C. K. E., Park, J., Kang, J., et al. (2019) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1904979116 · Cited by 82 · see citing works

Therapeutic Approaches to Treat Mitochondrial Diseases: “One-Size-Fits-All” and “Precision Medicine” Strategies

Bottani, E., Lamperti, C., Prigione, A., Tiranti, V., Persico, N., & Brunetti, D. (2020) · Pharmaceutics · doi:10.3390/pharmaceutics12111083 · Cited by 85 · see citing works

NAD+ as the Link Between Oxidative Stress, Inflammation, Caloric Restriction, Exercise, DNA Repair, Longevity, and Health Span

Poljšak, B., & Milisav, I. (2016) · Rejuvenation Research · doi:10.1089/rej.2015.1767 · Cited by 86 · see citing works

Mitochondrial Dysfunction in Age‐Related Metabolic Disorders

Natarajan, V., Chawla, R., Mah, T. K. L., Vivekanandan, R., Tan, S., Sato, P. Y., & Mallilankaraman, K. B. (2020) · PROTEOMICS · doi:10.1002/pmic.201800404 · Cited by 81 · see citing works

Nicotinamide riboside, a form of vitamin B3 and NAD+ precursor, relieves the nociceptive and aversive dimensions of paclitaxel-induced peripheral neuropathy in female rats

Hamity, M. V., White, S. R., Walder, R. Y., Schmidt, M. S., Brenner, C., & Hammond, D. L. (2017) · Pain · doi:10.1097/j.pain.0000000000000862 · Cited by 80 · see citing works

Muscle mitochondrial stress adaptation operates independently of endogenous FGF21 action

Ost, M., Coleman, V., Voigt, A., van Schothorst, E. M., Keipert, S., van der Stelt, I., Ringel, S., Graja, A., Ambrosi, T., Kipp, A. P., et al. (2016) · Molecular Metabolism · doi:10.1016/j.molmet.2015.11.002 · Cited by 75 · see citing works

BST1 regulates nicotinamide riboside metabolism via its glycohydrolase and base-exchange activities

Yaku, K., Palikhe, S., Izumi, H., Yoshida, T., Hikosaka, K., Hayat, F., Karim, M., Iqbal, T., Nitta, Y., Sato, A., et al. (2021) · Nature Communications · doi:10.1038/s41467-021-27080-3 · Cited by 76 · see citing works

Chatty Mitochondria: Keeping Balance in Cellular Protein Homeostasis

Topf, U., Wróbel, L., & Chacińska, A. (2016) · Trends in Cell Biology · doi:10.1016/j.tcb.2016.03.002 · Cited by 75 · see citing works

The Aging Metabolome—Biomarkers to Hub Metabolites

Sharma, R. B., & Ramanathan, A. (2020) · PROTEOMICS · doi:10.1002/pmic.201800407 · Cited by 76 · see citing works

NRH salvage and conversion to NAD+ requires NRH kinase activity by adenosine kinase

Yang, Y. J., Zhang, N., Zhang, G., & Sauve, A. A. (2020) · Nature Metabolism · doi:10.1038/s42255-020-0194-9 · Cited by 76 · see citing works

Moving towards clinical trials for mitochondrial diseases

Pitceathly, R. D. S., Keshavan, N., Rahman, J., & Rahman, S. (2020) · Journal of Inherited Metabolic Disease · doi:10.1002/jimd.12281 · Cited by 75 · see citing works

A need for NAD+ in muscle development, homeostasis, and aging

Goody, M. F., & Henry, C. A. (2018) · Skeletal Muscle · doi:10.1186/s13395-018-0154-1 · Cited by 75 · see citing works

Metabolomes of mitochondrial diseases and inclusion body myositis patients: treatment targets and biomarkers

Buzková, J., Nikkanen, J., Ahola, S., Hakonen, A. H., Sevastianova, K., Hovinen, T., Yki‐Järvinen, H., Pietiläinen, K. H., Lönnqvist, T. R. I., Velagapudi, V., et al. (2018) · EMBO Molecular Medicine · doi:10.15252/emmm.201809091 · Cited by 72 · see citing works

Reduced nicotinamide mononucleotide is a new and potent NAD + precursor in mammalian cells and mice

Perez, R. Z., Tammaro, A., Schomakers, B. V., Scantlebery, A. M. L., Denis, S. W., Elfrink, H. L., Giroud‐Gerbetant, J., Cantó, C., López‐Leonardo, C., McIntyre, R. L., et al. (2021) · The FASEB Journal · doi:10.1096/fj.202001826r · Cited by 75 · see citing works

Clinical trials in mitochondrial disorders, an update

Almannai, M., El‐Hattab, A. W., Ali, M. N., Soler‐Alfonso, C., & Scaglia, F. (2020) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2020.10.002 · Cited by 73 · see citing works

The virtuous cycle of human genetics and mouse models in drug discovery

Nadeau, J. H., & Auwerx, J. H. (2019) · Nature Reviews Drug Discovery · doi:10.1038/s41573-018-0009-9 · Cited by 71 · see citing works

Regulation of NAD biosynthetic enzymes modulates NAD-sensing processes to shape mammalian cell physiology under varying biological cues

Ruggieri, S., Orsomando, G., Sorci, L., & Raffaelli, N. (2015) · Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics · doi:10.1016/j.bbapap.2015.02.021 · Cited by 71 · see citing works

Keeping the balance in NAD metabolism

Strømland, Ø., Niere, M., Nikiforov, A. A., VanLinden, M. R., Heiland, I., & Ziegler, M. (2019) · Biochemical Society Transactions · doi:10.1042/bst20180417 · Cited by 72 · see citing works

NAD+ repletion with niacin counteracts cancer cachexia

Beltrà, M., Pöllänen, N., Fornelli, C., Tonttila, K., Hsu, M. Y., Zampieri, S., Moletta, L., Corrà, S., Porporato, P. E., Kivelä, R., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-37595-6 · Cited by 71 · see citing works

Mitochondrial diseases

Davis, R. L., Liang, C., & Sue, C. M. (2018) · Handbook of clinical neurology · doi:10.1016/b978-0-444-63233-3.00010-5 · Cited by 72 · see citing works

Mitochondrial DNA mutations in renal disease: an overview

Govers, L. P., Toka, H. R., Hariri, A., Walsh, S. B., & Böckenhauer, D. (2020) · Pediatric Nephrology · doi:10.1007/s00467-019-04404-6 · Cited by 70 · see citing works

The Unfolded Protein Responses in Health, Aging, and Neurodegeneration: Recent Advances and Future Considerations

Wodrich, A. P. K., Scott, A. W., Shukla, A. K., Harris, B. T., & Giniger, E. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2022.831116 · Cited by 66 · see citing works

Emerging therapies for mitochondrial disorders

Nightingale, H., Pfeffer, G., Bargiela, D., Horváth, R., & Chinnery, P. F. (2016) · Brain · doi:10.1093/brain/aww081 · Cited by 69 · see citing works

Research Article Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

Khan, N. A., Auranen, M., Paetau, I., Pirinen, E., Euro, L., Forsström, S., Pasila, L., Velagapudi, V., Carroll, C. J., Auwerx, J. H., et al. (2014)

Salvaging hope: Is increasing NAD+ a key to treating mitochondrial myopathy?

Lightowlers, R. N., & Chrzanowska‐Lightowlers, Z. M. (2014) · EMBO Molecular Medicine · doi:10.15252/emmm.201404179 · Cited by 17 · see citing works

Effects of nicotinamide riboside in mitochondrial DNA disease

Schon, K. R., Sleigh, A., Golder, Z. J., Berghella, A., Tiet, M. Y., Lyons, C., Bozhilova, L. V., Nie, Y., Harrison, E., Seikus, C., et al. (2026) · Genome Medicine · doi:10.1186/s13073-026-01764-1

NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease

Cerutti, R., Pirinen, E., Lamperti, C., Marchet, S., Sauve, A. A., Li, W., Leoni, V., Schon, E. A., Dantzer, F., Auwerx, J., et al. (2014) · Cell Metabolism · doi:10.1016/j.cmet.2014.04.001 · Cited by 343 · see citing works

Of Mice and Men: NAD+ Boosting with Niacin Provides Hope for Mitochondrial Myopathy Patients

Chini, E. N. (2020) · Cell Metabolism · doi:10.1016/j.cmet.2020.05.013 · Cited by 10 · see citing works