Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J. P., Deng, H.-X., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143

Methoden der enzymatischen Analyse

Bergmeyer, H. U. (1962) · Chemie eBooks · Cited by 4,030

Amyotrophic Lateral Ssclerosis and Structural Defects in Cu,Zn Superoxide Dismutase

Deng, H., Hentati, A., Tainer, J. A., Iqbal, Z., Cayabyab, A., Hung, W.-Y., Getzoff, E. D., Hu, P., Herzfeldt, B., Roos, R. P., et al. (1993) · Science · doi:10.1126/science.8351519 · Cited by 1,479

The control of flux.

KACSER, H., & Ja, B. (1973) · PubMed · Cited by 1,455

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

Wong, P. C., Pardo, C. A., Borchelt, D., Lee, M. K., Copeland, N. G., Jenkins, N. A., Sisodia, S. S., Cleveland, D. W., & Price, D. L. (1995) · Neuron · doi:10.1016/0896-6273(95)90259-7 · Cited by 1,439

Aging, energy, and oxidative stress in neurodegenerative diseases

Beal, M. F. (1995) · Annals of Neurology · doi:10.1002/ana.410380304 · Cited by 1,434

Diseases of the mitochondrial DNA

Wallace, D. C. (1992) · Annual Review of Biochemistry · doi:10.1146/annurev.bi.61.070192.005523 · Cited by 1,319

Role of uncoupled and non-coupled oxidations in maintenance of safely low levels of oxygen and its one-electron reductants

Skulachev, V. P. (1996) · Quarterly Reviews of Biophysics · doi:10.1017/s0033583500005795 · Cited by 703

Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis

Bowling, A. C., Schulz, J. B., Brown, R. H., & Beal, M. F. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb07478.x · Cited by 629

Molecular biology of amyotrophic lateral sclerosis: insights from genetics

Pasinelli, P., & Brown, R. H. (2006) · Nature Reviews Neuroscience · doi:10.1038/nrn1971 · Cited by 1,107

Amyotrophic lateral sclerosis

Wijesekera, L. C., & Leigh, P. N. (2009) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-4-3 · Cited by 1,130

Statin Adverse Effects: A Review of the Literature and Evidence for a Mitochondrial Mechanism

Golomb, B. A., & Evans, M. A. (2008) · American Journal of Cardiovascular Drugs · doi:10.2165/0129784-200808060-00004 · Cited by 677

The role of mitochondria in amyotrophic lateral sclerosis

Smith, E. F., Shaw, P. J., & Vos, K. J. D. (2017) · Neuroscience Letters · doi:10.1016/j.neulet.2017.06.052 · Cited by 579

Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: Benefit of a high-energy diet in a transgenic mouse model

Dupuis, L., Oudart, H., René, F., de Aguilar, J. G., & Loeffler, J. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0402026101 · Cited by 532

Energetics in the pathogenesis of neurodegenerative diseases

Beal, M. F. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01584-8 · Cited by 475

Mitochondrial Diseases of the Brain

Chaturvedi, R. K., & Beal, M. F. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.03.018 · Cited by 426

Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

Wiedemann, F. R., Manfredi, G., Mawrin, C., Beal, M. F., & Schon, E. A. (2002) · Journal of Neurochemistry · doi:10.1046/j.0022-3042.2001.00731.x · Cited by 383

Hypermetabolism in ALS patients: an early and persistent phenomenon

Bouteloup, C., Desport, J. C., Clavelou, P., Guy, N., Derumeaux-Burel, H., Ferrier, A., & Couratier, P. (2009) · Journal of Neurology · doi:10.1007/s00415-009-5100-z · Cited by 367

Increased lipid peroxidation in sera of ALS patients

Simpson, E. P., Henry, Y. K., Henkel-Tigges, J., Smith, R. G., & Appel, S. H. (2004) · Neurology · doi:10.1212/wnl.62.10.1758 · Cited by 328

Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice

Wong, M., & Martin, L. J. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq106 · Cited by 318

Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease

Albers, D. S., & Beal, M. F. (2000) · Springer eBooks · doi:10.1007/978-3-7091-6781-6_16 · Cited by 298

A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis

Zhao, Z., Lange, D. J., Voustianiouk, A., MacGrogan, D., Ho, L., Suh, J., Humala, N., Thiyagarajan, M., Wang, J., & Pasinetti, G. M. (2006) · BMC Neuroscience · doi:10.1186/1471-2202-7-29 · Cited by 292

A randomized sequential trial of creatine in amyotrophic lateral sclerosis

Groeneveld, G. J., Veldink, J. H., van der Tweel, I., Kalmijn, S., Beijer, C., de Visser, M., Wokke, J. H. J., Franssen, H., & van den Berg, L. H. (2003) · Annals of Neurology · doi:10.1002/ana.10554 · Cited by 282

Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis

Vielhaber, S. (2000) · Brain · doi:10.1093/brain/123.7.1339 · Cited by 268

Mitochondrial dysfunction in ALS

Cozzolino, M., & Carrì, M. T. (2011) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2011.06.003 · Cited by 249

Skeletal muscle mitochondria: A major player in exercise, health and disease

Russell, A. P., Foletta, V. C., Snow, R. J., & Wadley, G. D. (2013) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/j.bbagen.2013.11.016 · Cited by 233

Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis

Russell, A. P., Wada, S., Vergani, L., Hock, M. B., Lamon, S., Léger, B., Ushida, T., Cartoni, R., Wadley, G. D., Hespel, P., et al. (2012) · Neurobiology of Disease · doi:10.1016/j.nbd.2012.08.015 · Cited by 228

Oxidative Stress and Motor Neurone Disease

Cookson, M., & Shaw, P. J. (1999) · Brain Pathology · doi:10.1111/j.1750-3639.1999.tb00217.x · Cited by 223

Mitochondrial dysfunction and its role in motor neuron degeneration in ALS

Manfredi, G., & Xu, Z. (2005) · Mitochondrion · doi:10.1016/j.mito.2005.01.002 · Cited by 215

Mitochondrial involvement in amyotrophic lateral sclerosis

Menzies, F. M. (2002) · Neurochemistry International · doi:10.1016/s0197-0186(01)00125-5 · Cited by 209

Mitochondrial dysfunction, oxidative stress and neurodegeneration

Mancuso, M., Coppedè, F., Migliore, L., Siciliano, G., & Murri, L. (2006) · Journal of Alzheimer s Disease · doi:10.3233/jad-2006-10110 · Cited by 212

The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis

Loeffler, J., Picchiarelli, G., Dupuis, L., & de Aguilar, J. G. (2016) · Brain Pathology · doi:10.1111/bpa.12350 · Cited by 208

Resveratrol Improves Motoneuron Function and Extends Survival in SOD1G93A ALS Mice

Mancuso, R., del Valle, J., Mòdol, L., Martínez‐Muriana, A., Granado‐Serrano, A. B., Ramírez‐Núñez, O., Pallàs, M., Portero‐Otín, M., Osta, R., & Navarro, X. (2014) · Neurotherapeutics · doi:10.1007/s13311-013-0253-y · Cited by 212

ALS Pathogenesis and Therapeutic Approaches: The Role of Mesenchymal Stem Cells and Extracellular Vesicles

Bonafede, R., & Mariotti, R. (2017) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2017.00080 · Cited by 215

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis

Cudkowicz, M., Shefner, J. M., Schoenfeld, D., Brown, R. H., Jr., Johnson, H., Qureshi, M., Jacobs, M., Rothstein, J. D., Appel, S. H., Pascuzzi, R. M., et al. (2003) · Neurology · doi:10.1212/wnl.61.4.456 · Cited by 203

Mitochondria in Neurodegeneration

Lezi, E., & Swerdlow, R. H. (2011) · Advances in experimental medicine and biology · doi:10.1007/978-94-007-2869-1_12 · Cited by 213

Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis

Jung, C., Higgins, C. M. J., & Xu, Z. (2002) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2002.01112.x · Cited by 202

Autophagy and ALS: mechanistic insights and therapeutic implications

Chua, J. P., De Calbiac, H., Kabashi, E., & Barmada, S. J. (2022) · Autophagy · doi:10.1080/15548627.2021.1926656 · Cited by 189

Mitochondrial DNA damage and repair in neurodegenerative disorders

Yang, J., Weissman, L., Bohr, V. A., & Mattson, M. P. (2008) · DNA repair · doi:10.1016/j.dnarep.2008.03.012 · Cited by 187

Mitochondrial DNMT3A and DNA methylation in skeletal muscle and CNS of transgenic mouse models of ALS

Wong, M., Gertz, B., Chestnut, B. A., & Martin, L. J. (2013) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2013.00279 · Cited by 179

The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS)

Redler, R. L., & Dokholyan, N. V. (2012) · Progress in Molecular Biology and Translational Science · doi:10.1016/b978-0-12-385883-2.00002-3 · Cited by 172

Mitochondrial dysfunction and intracellular calcium dysregulation in ALS

Kawamata, H., & Manfredi, G. (2010) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2010.05.003 · Cited by 187

Interaction of Mitochondrial Calcium and ROS in Neurodegeneration

Baev, A. Y., Vinokurov, A. Y., Novikova, I. N., Dremin, V. V., Potapova, E. V., & Abramov, A. Y. (2022) · Cells · doi:10.3390/cells11040706 · Cited by 162

Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Implications

Cozzolino, M., Ferri, A., & Teresa Carrì, M. (2008) · Antioxidants & Redox Signaling · doi:10.1089/ars.2007.1760 · Cited by 156

High metabolic level in patients with familial amyotrophic lateral sclerosis

Funalot, B., Desport, J., Sturtz, F. G., Camu, W., & Couratier, P. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802295192 · Cited by 153

Cytoplasmic hybrid (cybrid) cell lines as a practical model for mitochondriopathies

Wilkins, H., Carl, S. M., & Swerdlow, R. H. (2014) · Redox Biology · doi:10.1016/j.redox.2014.03.006 · Cited by 152

Mitochondria in Amyotrophic Lateral Sclerosis: A Trigger and a Target

Dupuis, L., de Aguilar, J. G., Oudart, H., de Tapia, M., Barbeito, L., & Loeffler, J. (2004) · Neurodegenerative Diseases · doi:10.1159/000085063 · Cited by 157

Effects of high-intensity endurance exercise training in the G93A mouse model of amyotrophic lateral sclerosis

Mahoney, D. J., Rodriguez, C., Devries, M., Yasuda, N., & Tarnopolsky, M. A. (2004) · Muscle & Nerve · doi:10.1002/mus.20004 · Cited by 140

Mitochondria and Neurodegeneration

Petrozzi, L., Ricci, G., Giglioli, N. J., Siciliano, G., & Mancuso, M. (2007) · Bioscience Reports · doi:10.1007/s10540-007-9038-z · Cited by 139

Caprylic Triglyceride as a Novel Therapeutic Approach to Effectively Improve the Performance and Attenuate the Symptoms Due to the Motor Neuron Loss in ALS Disease

Zhao, W., Varghese, M., Vempati, P., Dzhun, A., Cheng, A., Wang, J., Lange, D. J., Bilski, A. E., Faravelli, I., & Pasinetti, G. M. (2012) · PLoS ONE · doi:10.1371/journal.pone.0049191 · Cited by 135

Mitochondria in cybrids containing mtDNA from persons with mitochondriopathies

Swerdlow, R. H. (2007) · Journal of Neuroscience Research · doi:10.1002/jnr.21167 · Cited by 128

Mitochondrial dynamics, a key executioner in neurodegenerative diseases

Panchal, K., & Tiwari, A. K. (2018) · Mitochondrion · doi:10.1016/j.mito.2018.11.002 · Cited by 128

Glucose Metabolic Dysfunction in Neurodegenerative Diseases—New Mechanistic Insights and the Potential of Hypoxia as a Prospective Therapy Targeting Metabolic Reprogramming

Han, R., Liang, J., & Zhou, B. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22115887 · Cited by 125

Mitochondrial dysfunction and amyotrophic lateral sclerosis

Hervías, I., Beal, M. F., & Manfredi, G. (2005) · Muscle & Nerve · doi:10.1002/mus.20489 · Cited by 122

Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis

Vielhaber, S., Winkler, K., Kirches, E., Kunz, D., Büchner, M., Feistner, H., Elger, C. E., Ludolph, A. C., Riepe, M. W., & Kunz, W. S. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00236-1 · Cited by 121

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

Mancuso, R., & Navarro, X. (2015) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2015.07.004 · Cited by 125

The Neurodegenerative Mitochondriopathies

Swerdlow, R. H. (2009) · Journal of Alzheimer s Disease · doi:10.3233/jad-2009-1095 · Cited by 115

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

Jaiswal, M. K., Zech, W., Goos, M., Leutbecher, C., Ferri, A., Zippelius, A., Carrı̀, M. T., Nau, R., & Keller, B. U. (2009) · BMC Neuroscience · doi:10.1186/1471-2202-10-64 · Cited by 111

Are oxidative stress and mitochondrial dysfunction the key players in the neurodegenerative diseases?

Carlo, M. D., Giacomazza, D., Picone, P., Nuzzo, D., & Biagio, P. L. S. (2012) · Free Radical Research · doi:10.3109/10715762.2012.714466 · Cited by 106

Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis

Allen, S. P., Hall, B., Castelli, L. M., Francis, L. I., Woof, R., Siskos, A., Kouloura, E., Gray, E., Thompson, A. G., Talbot, K., et al. (2019) · Brain · doi:10.1093/brain/awy353 · Cited by 102

Multifaceted deaths orchestrated by mitochondria in neurones

Nagley, P., Higgins, G. C., Atkin, J. D., & Beart, P. M. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2009.09.004 · Cited by 107

A fruitful endeavor: Modeling ALS in the fruit fly

Casci, I., & Pandey, U. B. (2014) · Brain Research · doi:10.1016/j.brainres.2014.09.064 · Cited by 102

The Role of Mitochondria in the Pathogenesis of Neurodegenerative Diseases

Manfredim, G., & Beal, M. F. (2000) · Brain Pathology · doi:10.1111/j.1750-3639.2000.tb00278.x · Cited by 101

Genetic epidemiology of amyotrophic lateral sclerosis

Majoor‐Krakauer, D., Willems, P., & Hofman, A. (2003) · Clinical Genetics · doi:10.1046/j.0009-9163.2002.00001.x · Cited by 147

Decreased mRNA Expression of PGC-1α and PGC-1α-Regulated Factors in the SOD1G93AALS Mouse Model and in Human Sporadic ALS

Thau, N., Knippenberg, S., Körner, S., Rath, K. J., Dengler, R., & Petri, S. (2012) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e318275df4b · Cited by 97

Amyotrophic Lateral Sclerosis and Autophagy: Dysfunction and Therapeutic Targeting

Amin, A., Perera, N. D., Beart, P. M., Turner, B. J., & Shabanpoor, F. (2020) · Cells · doi:10.3390/cells9112413 · Cited by 103

Skeletal-Muscle Metabolic Reprogramming in ALS-SOD1G93A Mice Predates Disease Onset and Is A Promising Therapeutic Target

Scaricamazza, S., Salvatori, I., Giacovazzo, G., Loeffler, J., René, F., Rosina, M., Quessada, C., Proietti, D., Heil, C., Rossi, S., et al. (2020) · iScience · doi:10.1016/j.isci.2020.101087 · Cited by 98

The basic aspects of therapeutics in amyotrophic lateral sclerosis

Strong, M. J. (2003) · Pharmacology & Therapeutics · doi:10.1016/s0163-7258(03)00040-8 · Cited by 94

Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies

Krasnianski, A., Deschauer, M., Neudecker, S., Gellerich, F. N., Müller, T., Schoser, B. G. H., Krasnianski, M., & Zierz, S. (2005) · Brain · doi:10.1093/brain/awh540 · Cited by 94

Increased SOD1 association with chromatin, DNA damage, p53 activation, and apoptosis in a cellular model of SOD1-linked ALS

Barbosa, L. F., Cerqueira, F. M., Macedo, A. F. D. A., Garcia, C. C. M., Angeli, J. P. F., Schumacher, R. I., Sogayar, M. C., Augusto, O., Carrı̀, M. T., Mascio, P. D., et al. (2010) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2010.01.011 · Cited by 88

Strictly monitored exercise programs reduce motor deterioration in ALS: preliminary results of a randomized controlled trial

Lunetta, C., Lizio, A., Sansone, V., Cellotto, N. M., Maestri, E., Bettinelli, M., Gatti, V., Melazzini, M. G., Meola, G., & Corbo, M. (2015) · Journal of Neurology · doi:10.1007/s00415-015-7924-z · Cited by 98

Expression of mutant SOD1 G93A in astrocytes induces functional deficits in motoneuron mitochondria

Bilsland, L. G., Nirmalananthan, N., Yip, J., Greensmith, L., & Duchen, M. R. (2008) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2008.05699.x · Cited by 88

Abnormalities of satellite cells function in amyotrophic lateral sclerosis

Pradat, P., Barani, A. E., Wanschitz, J., Dubourg, O., Lombès, A., Bigot, A., Mouly, V., Bruneteau, G., Salachas, F., Lenglet, T., et al. (2011) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482968.2011.566618 · Cited by 88

Metabolic Reprogramming in Amyotrophic Lateral Sclerosis

Szelechowski, M., Amoêdo, N. D., Obre, É., Leger, C., Allard, L., Bonneu, M., Claverol, S., Lacombe, D., Oliet, S. H. R., Chevallier, S., et al. (2018) · Scientific Reports · doi:10.1038/s41598-018-22318-5 · Cited by 84

The role of excitotoxicity in ALS - what is the evidence?

Ludolph, A. C., Meyer, T., & Riepe, M. W. (2000) · Journal of Neurology · doi:10.1007/s004150050552 · Cited by 84

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

Tradewell, M. L., Cooper, L. A., Minotti, S., & Durham, H. D. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.01.016 · Cited by 81

ASCs-Exosomes Recover Coupling Efficiency and Mitochondrial Membrane Potential in an in vitro Model of ALS

Calabria, E., Scambi, I., Bonafede, R., Schiaffino, L., Peroni, D., Potrich, V., Capelli, C., Schena, F. F., & Mariotti, R. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.01070 · Cited by 81

Redox Regulation in Amyotrophic Lateral Sclerosis

Parakh, S., Spencer, D. M. S., Halloran, M. A., Soo, K. Y., & Atkin, J. D. (2013) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2013/408681 · Cited by 85

Mitochondria and the pathogenesis of ALS

Beal, M. F. (2000) · Brain · doi:10.1093/brain/123.7.1291 · Cited by 80

What is “Hyper” in the ALS Hypermetabolism?

Ferri, A., & Coccurello, R. (2017) · Mediators of Inflammation · doi:10.1155/2017/7821672 · Cited by 77

Coenzyme Q treatment of neurodegenerative diseases of aging

Galpern, W. R., & Cudkowicz, M. (2007) · Mitochondrion · doi:10.1016/j.mito.2007.01.004 · Cited by 78

Oxidative stress and antioxidant enzyme upregulation in SOD1‐G93A mouse skeletal muscle

Mahoney, D. J., Kaczor, J. J., Bourgeois, J. M., Yasuda, N., & Tarnopolsky, M. A. (2006) · Muscle & Nerve · doi:10.1002/mus.20542 · Cited by 80

Effect of coenzyme Q10 on the mitochondrial function of skin fibroblasts from Parkinson patients

Winkler‐Stuck, K., Wiedemann, F. R., Wallesch, C., & Kunz, W. S. (2004) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2004.02.003 · Cited by 77

Calcium: the Darth Vader of ALS

Appel, S. H., Beers, D. R., Siklós, L., Engelhardt, J., & Mosier, D. R. (2001) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608201300079418 · Cited by 88

PGC‐1α protects neurons and alters disease progression in an amyotrophic lateral sclerosis mouse model

Liang, H., Ward, W. F., Jang, Y. C., Bhattacharya, A., Bokov, A. F., Li, Y., Jernigan, A., Richardson, A., & Remmen, H. V. (2011) · Muscle & Nerve · doi:10.1002/mus.22217 · Cited by 78

Mitochondrial Dysfunction in Neuromuscular Disorders

Katsetos, C. D., Koutzaki, S. H., & Melvin, J. J. (2013) · Seminars in Pediatric Neurology · doi:10.1016/j.spen.2013.10.010 · Cited by 75

Cytochrome c Oxidase and Its Role in Neurodegeneration and Neuroprotection

Arnold, S. (2012) · Advances in experimental medicine and biology · doi:10.1007/978-1-4614-3573-0_13 · Cited by 77

Mitochondrial respiratory chain function in skeletal muscle of ALS patients

Echaniz‐Laguna, A., Zoll, J., Ribera, F., Tranchant, C., Warter, J., Lonsdorfer, J., & Lampert, E. (2002) · Annals of Neurology · doi:10.1002/ana.10357 · Cited by 75

Manganese‐containing superoxide dismutase signal sequence polymorphism associated with sporadic motor neuron disease

van Landeghem, G. F., Tabatabaie, P., Beckman, G., Beckman, L. E., & Andersen, P. M. (1999) · European Journal of Neurology · doi:10.1046/j.1468-1331.1999.660639.x · Cited by 75

Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure

Panes-Fernández, J., Wendt, A., Ramírez-Molina, O., Castro, P. A., & Fuentealba, J. (2021) · Neural Regeneration Research · doi:10.4103/1673-5374.317957 · Cited by 77

Mitochondrial dysfunction in neurodegenerative disorders

Baron, M. S., Kudin, A. P., & Kunz, W. S. (2007) · Biochemical Society Transactions · doi:10.1042/bst0351228 · Cited by 74

Progress in Clinical Neurosciences: The Evidence for ALS as a Multisystems Disorder of Limited Phenotypic Expression

Strong, M. J. (2001) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100001505 · Cited by 73

l-Carnitine suppresses the onset of neuromuscular degeneration and increases the life span of mice with familial amyotrophic lateral sclerosis

Kira, Y., Nishikawa, M., Ochi, A., Sato, E. F., & Inoue, M. (2006) · Brain Research · doi:10.1016/j.brainres.2005.11.052 · Cited by 70

Age‐related decreases in NAD(P)H and glutathione cause redox declines before ATP loss during glutamate treatment of hippocampal neurons

Parihar, M. S., Kunz, E. A., & Brewer, G. J. (2008) · Journal of Neuroscience Research · doi:10.1002/jnr.21679 · Cited by 69

Nutritional and metabolic support in patients with amyotrophic lateral sclerosis

Muscaritoli, M., Kushta, I., Molfino, A., Inghilleri, M., Sabatelli, M., & Fanelli, F. R. (2012) · Nutrition · doi:10.1016/j.nut.2012.01.011 · Cited by 70

Role of platelets in neurodegenerative diseases: a universal pathophysiology

Behari, M., & Shrivastava, M. (2013) · International Journal of Neuroscience · doi:10.3109/00207454.2012.751534 · Cited by 68

Could mitochondrial haplogroups play a role in sporadic amyotrophic lateral sclerosis?

Mancuso, M., Conforti, F. L., Rocchi, A., Tessitore, A., Muglia, M., Tedeschi, G., Panza, D., Monsurrò, M., Sola, P., Mandrioli, J., et al. (2004) · Neuroscience Letters · doi:10.1016/j.neulet.2004.08.060 · Cited by 67

More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases

Boyer, J. G., Ferrier, A., & Kothary, R. (2013) · Frontiers in Physiology · doi:10.3389/fphys.2013.00356 · Cited by 69

Mitochondrial abnormalities in muscle and other aging cells: Classification, causes, and effects

DiMauro, S., Tanji, K., Bonilla, E., Pallotti, F., & Schon, E. A. (2002) · Muscle & Nerve · doi:10.1002/mus.10194 · Cited by 64

Mitochondrial complex I deficiency and ATP/ADP ratio in lymphocytes of amyotrophic lateral sclerosis patients

Ghiasi, P., Hosseinkhani, S., Noori, A.-R., Nafissi, S., & Khajeh, K. (2012) · Neurological Research · doi:10.1179/1743132812y.0000000012 · Cited by 64

Mitochondrial Involvement in Amyotrophic Lateral Sclerosis: Trigger or Target?

Bacman, S. R., Bradley, W. G., & Moraes, C. T. (2006) · Molecular Neurobiology · doi:10.1385/mn:33:2:113 · Cited by 66

Therapeutic progress in amyotrophic lateral sclerosis-beginning to learning

Kumar, V., Islam, A., Hassan, M. I., & Ahmad, F. (2016) · European Journal of Medicinal Chemistry · doi:10.1016/j.ejmech.2016.06.017 · Cited by 69

Re-evaluation of the dysfunction of mitochondrial respiratory chain in skeletal muscle of patients with Parkinson?s disease

Winkler-Stuck, K., Kirches, E., Mawrin, C., Dietzmann, K., Lins, H., Wallesch, C.-W., Kunz, W. S., & Wiedemann, F. R. (2005) · Journal of Neural Transmission · doi:10.1007/s00702-004-0195-y · Cited by 63

Metabolic and Stress Response Changes Precede Disease Onset in the Spinal Cord of Mutant SOD1 ALS Mice

Pharaoh, G. A., Sataranatarajan, K., Street, K., Hill, S., Gregston, J., Ahn, B., Kinter, C. S., Kinter, M. T., & Remmen, H. V. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.00487 · Cited by 64

Effects of mitochondrial dysfunction on glutamate receptor-mediated neurotoxicity in cultured rat spinal motor neurons

Kanki, R., Nakamizo, T., Yamashita, H., Kihara, T., Sawada, H., Uemura, K., Kawamata, J., Shibasaki, H., Akaike, A., & Shimohama, S. (2004) · Brain Research · doi:10.1016/j.brainres.2004.04.044 · Cited by 62

Dysregulated mitochondrial Ca2+ and ROS signaling in skeletal muscle of ALS mouse model

Zhou, J., Li, A., Li, X., & Yi, J. (2019) · Archives of Biochemistry and Biophysics · doi:10.1016/j.abb.2019.01.024 · Cited by 59

Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral Sclerosis

Tefera, T. W., Wong, Y., Barkl‐Luke, M. E., Ngo, S. T., Thomas, N. K., McDonald, T. S., & Borges, K. (2016) · PLoS ONE · doi:10.1371/journal.pone.0161816 · Cited by 61

G37R SOD1 mutant alters mitochondrial complex I activity, Ca2+ uptake and ATP production

Coussée, E., Smet, P. D., Bogaert, E., Elens, I., Damme, P. V., Willems, P. H. G. M., Koopman, W. J. H., Bosch, L. V. D., & Callewaert, G. (2011) · Cell Calcium · doi:10.1016/j.ceca.2011.02.004 · Cited by 58

Skeletal Muscle Satellite Cells, Mitochondria, and MicroRNAs: Their Involvement in the Pathogenesis of ALS

Tsitkanou, S., Gatta, P. A. D., & Russell, A. P. (2016) · Frontiers in Physiology · doi:10.3389/fphys.2016.00403 · Cited by 57

Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis

Vielhaber, S. (2000) · Brain · doi:10.1093/brain/123.7.1339 · Cited by 268

Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis

Vielhaber, S., Winkler, K., Kirches, E., Kunz, D., Büchner, M., Feistner, H., Elger, C. E., Ludolph, A. C., Riepe, M. W., & Kunz, W. S. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00236-1 · Cited by 121

Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis

Crugnola, V., Lamperti, C., Lucchini, V., Ronchi, D., Peverelli, L., Prelle, A. C., Sciacco, M., Bordoni, A., Fassone, E., Fortunato, F., et al. (2010) · Archives of Neurology · doi:10.1001/archneurol.2010.128 · Cited by 152

Mitochondrial dysfunction in amyotrophic lateral sclerosis also affects skeletal muscle

Dupuis, L., de Aguilar, J. G., Echaniz‐Laguna, A., & Loeffler, J. (2006) · Muscle & Nerve · doi:10.1002/mus.20566 · Cited by 17

Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies

Krasnianski, A., Deschauer, M., Neudecker, S., Gellerich, F. N., Müller, T., Schoser, B. G. H., Krasnianski, M., & Zierz, S. (2005) · Brain · doi:10.1093/brain/awh540 · Cited by 94