The DOI matches its Crossref record; 26 of the 27 references it cites were found in a registry or an index.
Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis
Abstract
In skeletal muscle homogenates of 14 patients with sporadic amyotrophic lateral sclerosis, an approximately twofold lower specific activity of NADH:CoQ oxidoreductase in comparison to an age matched control group (n=28) was detected. This finding was confirmed by a detailed analysis of mitochondrial oxidative phosphorylation in skeletal muscle using saponin-permeabilized muscle fibers. (i) A significantly lowered maximal glutamate+malate and pyruvate+malate supported respiration of saponin-permeabilized fibers was detected in the patients group. (ii) Titrations with the specific inhibitor of NADH:CoQ oxidoreductase amytal revealed a higher sensitivity of respiration to this inhibitor indicating an elevated flux control coefficient of this enzyme. (iii) Applying functional imaging of mitochondria using ratios of NAD(P)H and flavoprotein autofluorescence images of saponin-permeabilized fibers we detected the presence of partially respiratory chain inhibited mitochondria on the single fiber level. A secondary defect of mitochondrial function due to the neurogenic changes in muscle seems to be unlikely since no mitochondrial abnormalities were detectable in biopsies of patients with spinal muscular atrophy. These results support the viewpoint that an impairment of mitochondria may be of pathophysiological significance in the etiology of amyotrophic lateral sclerosis.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“Accumulations of dysmorphic and enlarged mitochondria, many with deficiencies of critical oxidative enzymes, in anterior horn cells, intramuscular nerves, and skeletal muscle are characteristic of sALS [281–285].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [285]
Citations
Cites
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
KACSER, H., & Ja, B. (1973) · PubMed · Cited by 1,455
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
Skulachev, V. P. (1996) · Quarterly Reviews of Biophysics · doi:10.1017/s0033583500005795 · Cited by 703
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
Cited by
Sorted by citations
Items 101–200 of 262
Vitamin D as a Potential Therapy in Amyotrophic Lateral Sclerosis
Gianforcaro, A., & Hamadeh, M. J. (2014) · CNS Neuroscience & Therapeutics · doi:10.1111/cns.12204 · Cited by 61
Singh, T., Jiao, Y., Ferrando, L. M., Yablonska, S., Li, F., Horoszko, E. C., Lacomis, D., Friedlander, R. M., & Carlisle, D. L. (2021) · Scientific Reports · doi:10.1038/s41598-021-97928-7 · Cited by 55
Labarre, A., Guitard, E., Tossing, G., Forest, A., Bareke, E., Labrecque, M., Tétreault, M., Ruiz, M., & Parker, J. A. (2022) · Communications Biology · doi:10.1038/s42003-022-04295-8 · Cited by 55
Al‐Sarraj, S., King, A., Cleveland, M. S., Pradat, P., Corse, A. M., Rothstein, J. D., Leigh, P., Abila, B., Bates, S., Würthner, J., et al. (2014) · Acta Neuropathologica Communications · doi:10.1186/s40478-014-0165-z · Cited by 57
Antioxidant capacity and protein oxidation in cerebrospinal fluid of amyotrophic lateral sclerosis
Siciliano, G., Piazza, S., Carlesi, C., Corona, A., Franzini, M., Alfonso Pompella, C., Malvaldi, G., Mancuso, M., Paolicchi, A., & Murri, L. (2007) · Journal of Neurology · doi:10.1007/s00415-006-0301-1 · Cited by 55
Jaiswal, M. K. (2014) · Molecular and Cellular Therapies · doi:10.1186/2052-8426-2-26 · Cited by 56
Magnetic resonance spectroscopy reveals mitochondrial dysfunction in amyotrophic lateral sclerosis
Sassani, M., Alix, J. J. P., McDermott, C. J., Baster, K., Hoggard, N., Wild, J. M., Mortiboys, H., Shaw, P. J., Wilkinson, I. D., & Jenkins, T. M. (2020) · Brain · doi:10.1093/brain/awaa340 · Cited by 53
AMPK Signalling and Defective Energy Metabolism in Amyotrophic Lateral Sclerosis
Perera, N. D., & Turner, B. J. (2015) · Neurochemical Research · doi:10.1007/s11064-015-1665-3 · Cited by 51
Martin, L. J., & Wong, M. (2020) · Frontiers in Neurology · doi:10.3389/fneur.2020.592851 · Cited by 49
Effect of Creatine Supplementation on Metabolite Levels in ALS Motor Cortices
Vielhaber, S., Kaufmann, J., Kanowski, M., Sailer, M., Feistner, H., Tempelmann, C., Elger, C. E., Heinze, H., & Kunz, W. S. (2001) · Experimental Neurology · doi:10.1006/exnr.2001.7797 · Cited by 49
Self-citationMitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system
Bertamini, M., Marzani, B., Guarneri, R., Guarneri, P., Bigini, P., Mennini, T., & Curti, D. (2002) · European Journal of Neuroscience · doi:10.1046/j.1460-9568.2002.02299.x · Cited by 48
Ngo, S. T., & Steyn, F. J. (2015) · Cell Regeneration · doi:10.1186/s13619-015-0019-6 · Cited by 46
Chowdhury, S. R., Djordjevic, J., Albensi, B. C., & Fernyhough, P. (2015) · Bioscience Reports · doi:10.1042/bsr20150244 · Cited by 46
Bahadorani, S., Mukai, S. T., Rabie, J., Beckman, J. S., Phillips, J. P., & Hilliker, A. J. (2013) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2013.03.024 · Cited by 45
Pehar, M., Beeson, G. C., Beeson, C., Johnson, J. A., & Vargas, M. R. (2014) · PLoS ONE · doi:10.1371/journal.pone.0103438 · Cited by 44
Dave, K. R., Prado, R., Busto, R., Raval, A. P., Bradley, W. G., Torbati, D., & Pérez‐Pinzón, M. A. (2003) · Neuroscience · doi:10.1016/s0306-4522(03)00244-6 · Cited by 44
Leclerc, N., Ribera, F., Zoll, J., Warter, J., Poindron, P., Lampert, E., & Borg, J. (2001) · Neuromuscular Disorders · doi:10.1016/s0960-8966(01)00240-1 · Cited by 44
TDP-43 dysregulation and neuromuscular junction disruption in amyotrophic lateral sclerosis
Lépine, S., Castellanos‐Montiel, M. J., & Durcan, T. M. (2022) · Translational Neurodegeneration · doi:10.1186/s40035-022-00331-z · Cited by 46
Skeletal muscle fiber function and rate of disease progression in amyotrophic lateral sclerosis
Krivickas, L. S., Yang, J., Kim, S., & Frontera, W. R. (2002) · Muscle & Nerve · doi:10.1002/mus.10257 · Cited by 43
Serum levels of coenzyme Q 10 in patients with amyotrophic lateral sclerosis
Molina, J. A., de Bustos, F., Jiménez‐Jiménez, F. J., Gómez-Escalonilla, C., García‐Redondo, A., Esteban, J., Guerrero-Sola, A., del Hoyo, P., Martínez‐Salio, A., Ramírez-Ramos, C., et al. (2000) · Journal of Neural Transmission · doi:10.1007/s007020070050 · Cited by 43
Impaired oxidative metabolism in exercising muscle from ALS patients
Siciliano, G., Pastorini, E., Pasquali, L., Manca, M. L., Iudice, A., & Murri, L. (2001) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(01)00620-7 · Cited by 43
Kupsch, A. R., Schmidt, W., Gizatullina, Z. Z., Dębska–Vielhaber, G., Voges, J., Striggow, F., Panther, P., Schwegler, H., Heinze, H., Vielhaber, S., et al. (2014) · Journal of Neural Transmission · doi:10.1007/s00702-014-1185-3 · Cited by 42
Self-citationAmyotrophic lateral sclerosis linked to a novel SOD1 mutation with muscle mitochondrial dysfunction
Corti, S. P., Donadoni, C., Ronchi, D., Bordoni, A., Fortunato, F., Santoro, D., Bo, R. D., Lucchini, V., Crugnola, V., Papadimitriou, D. A., et al. (2008) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2008.09.030 · Cited by 41
Role of mitochondria in amyotrophic lateral sclerosis
Swerdlow, R. H., Parks, J. K., Pattee, G. L., & Parker, W. D. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820050515179 · Cited by 41
Shrivastava, M., Vivekanandhan, S., Pati, U., Behari, M., & Das, T. K. (2010) · International Journal of Neuroscience · doi:10.3109/00207454.2010.537416 · Cited by 40
Flis, D. J., Dzik, K. P., Kaczor, J. J., Hałoń, M., Antosiewicz, J., Wieckowski, M. R., & Ziółkowski, W. (2018) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2018/5940748 · Cited by 39
Badu-Mensah, A., Guo, X., McAleer, C. W., Rumsey, J. W., & Hickman, J. J. (2020) · Scientific Reports · doi:10.1038/s41598-020-70510-3 · Cited by 39
de Aguilar, J. G., Dupuis, L., Oudart, H., & Loeffler, J. (2005) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2005.03.003 · Cited by 39
Belosludtseva, N. V., Matveeva, L. A., & Belosludtsev, K. N. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms242316833 · Cited by 40
Golomb, B. A., Verden, A., Messner, A. K., Koslik, H. J., & Hoffman, K. B. (2018) · Drug Safety · doi:10.1007/s40264-017-0620-4 · Cited by 38
Interplay between TDP-43 and docosahexaenoic acid-related processes in amyotrophic lateral sclerosis
Cacabelos, D., Ayala, V., Granado‐Serrano, A. B., Jové, M., Torres, P., Boada, J., Cabré, R., Ramírez‐Núñez, O., Gonzalo‐Benito, H., Soler-Cantero, A., et al. (2016) · Neurobiology of Disease · doi:10.1016/j.nbd.2016.01.007 · Cited by 39
Synaptic Failure: Focus in an Integrative View of ALS
Casas, C., Manzano, R., Vaz, A. R., Osta, R., & Brites, D. M. T. O. (2015) · Brain Plasticity · doi:10.3233/bpl-140001 · Cited by 39
Mayevsky, A., & Barbiro‐Michaely, E. (2012) · Journal of Clinical Monitoring and Computing · doi:10.1007/s10877-012-9413-6 · Cited by 37
Reduction of oxidative stress in amyotrophic lateral sclerosis following pramipexole treatment
Pattee, G. L., Post, G. R., Gerber, R. E., & Bennett, J. P. (2003) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820310012736 · Cited by 36
Retinal flavoprotein autofluorescence as a measure of retinal health.
Elner, S. G., Elner, V. M., Field, M. G., Park, S. L., Heckenlively, J. R., & Petty, H. R. (2008) · PubMed Central · Cited by 36
H. Swerdlow, R. (2011) · Current Pharmaceutical Design · doi:10.2174/138161211798072535 · Cited by 35
New Therapeutics to Modulate Mitochondrial Function in Neurodegenerative Disorders
Wilkins, H., & Morris, J. K. (2017) · Current Pharmaceutical Design · doi:10.2174/1381612822666161230144517 · Cited by 34
What skeletal muscle has to say in amyotrophic lateral sclerosis: Implications for therapy
Manzano, R., Toivonen, J. M., Moreno‐Martínez, L., de la Torre, M., Moreno‐García, L., López‐Royo, T., Molina, N., Zaragoza, P., Calvo, A. C., & Osta, R. (2020) · British Journal of Pharmacology · doi:10.1111/bph.15276 · Cited by 34
Smittkamp, S. E., Spalding, H. N., Brown, J. W., Gupte, A. A., Chen, J., Nishimune, H., Geiger, P. C., & Stanford, J. A. (2010) · Behavioural Brain Research · doi:10.1016/j.bbr.2010.03.007 · Cited by 35
Dysfunctional mitochondrial respiration in the wobbler mouse brain
Xu, G.-P., Dave, K. R., Moraes, C. T., Busto, R., Sick, T. J., Bradley, W. G., & Pérez‐Pinzón, M. A. (2001) · Neuroscience Letters · doi:10.1016/s0304-3940(01)01575-0 · Cited by 34
Janus kinase inhibitors are potential therapeutics for amyotrophic lateral sclerosis
Richardson, P. J., Smith, D. P., de Giorgio, A., Snetkov, X., Almond-Thynne, J., Cronin, S., Mead, R. J., McDermott, C. J., & Shaw, P. J. (2023) · Translational Neurodegeneration · doi:10.1186/s40035-023-00380-y · Cited by 35
Hayes, L. R., Brotherton, T. E., & Glass, J. D. (2013) · Experimental Neurology · doi:10.1016/j.expneurol.2013.05.001 · Cited by 37
Potential benefits of medium chain fatty acids in aging and neurodegenerative disease
Dunn, E., Zhang, B., Sahota, V. K., & Augustin, H. (2023) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2023.1230467 · Cited by 38
Translating preclinical insights into effective human trials in ALS
DiBernardo, A., & Cudkowicz, M. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.007 · Cited by 34
N’guessan, B. B., Zoll, J., Ribera, F., Ponsot, E., Lampert, E., Ventura‐Clapier, R., Veksler, V., & Mettauer, B. (2003) · Molecular and Cellular Biochemistry · doi:10.1023/b:mcbi.0000009874.14649.ca · Cited by 33
Dave, K. R., Bradley, W. G., & Pérez‐Pinzón, M. A. (2003) · Experimental Neurology · doi:10.1016/s0014-4886(03)00091-8 · Cited by 34
Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity
McCombe, P., Wray, N. R., & Henderson, R. D. (2016) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2017.1273772 · Cited by 35
Mitochondria: A Promising Convergent Target for the Treatment of Amyotrophic Lateral Sclerosis
Cunha‐Oliveira, T., Montezinho, L., Simões, R. F., de Carvalho, M., Ferreiro, E. B., & Silva, F. S. G. (2024) · Cells · doi:10.3390/cells13030248 · Cited by 34
Biomarkers of Metabolism in Amyotrophic Lateral Sclerosis
Kirk, S. E., Tracey, T. J., Steyn, F. J., & Ngo, S. T. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00191 · Cited by 38
Quessada, C., Bouscary, A., René, F., Valle, C., Ferri, A., Ngo, S. T., & Loeffler, J. (2021) · Cells · doi:10.3390/cells10061449 · Cited by 33
Abu‐Hamad, S., Kahn, J., Leyton-Jaimes, M. F., Rosenblatt, J. D., & Israelson, A. (2017) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.7b00140 · Cited by 32
Ngo, S. T., Mi, J. D., Henderson, R. D., McCombe, P., & Steyn, F. J. (2017) · Degenerative Neurological and Neuromuscular Disease · doi:10.2147/dnnd.s120607 · Cited by 31
Altered Bioenergetics and Metabolic Homeostasis in Amyotrophic Lateral Sclerosis
Nelson, A. T., & Trotti, D. (2022) · Neurotherapeutics · doi:10.1007/s13311-022-01262-3 · Cited by 31
Gajewski, C. D., Lin, M. T. S., Cudkowicz, M., Beal, M. F., & Manfredi, G. (2003) · Experimental Neurology · doi:10.1016/s0014-4886(02)00022-5 · Cited by 30
Activities of mitochondrial complexes correlate with nNOS amount in muscle from ALS patients
Sorarú, G., Vergani, L., Fedrizzi, L., D’Ascenzo, C., Polo, A., Bernazzi, B., & Angelini, C. I. (2007) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2006.00791.x · Cited by 29
Neurodegenerative disorders, metabolic icebergs, and mitohormesis
Phillips, M. C. L., & Picard, M. A. (2024) · Translational Neurodegeneration · doi:10.1186/s40035-024-00435-8 · Cited by 31
Metabolic Consequences of the CytochromecOxidase Deficiency in Brain of Copper‐Deficient MovbrMice
Kunz, W. S., Kuznetsov, A. V., Clark, J. F., Tracey, I., & Elger, C. E. (1999) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1999.721580.x · Cited by 28
Self-citationDębska–Vielhaber, G., Miller, I., Peeva, V., Zuschratter, W., Walczak, J., Schreiber, S., Petri, S., Machts, J., Vogt, S., Szczepanowska, J., et al. (2021) · Experimental Neurology · doi:10.1016/j.expneurol.2021.113620 · Cited by 28
Self-citationPalencia, P., QUIROZ‐ROTHE, E., & Rivero, J. L. L. (2004) · Acta Neuropathologica · doi:10.1007/s00401-004-0940-1 · Cited by 26
The "new" mitochondrial disorders
Schapira, A. H. V. (2002) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.72.2.144 · Cited by 26
Wiedemann, F. R., Winkler, K., Lins, H., Wallesch, C., & Kunz, W. S. (1999) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1999.tb07870.x · Cited by 25
Self-citationLanznaster, D., Bruno, C., Bourgeais, J., Emond, P., Zemmoura, I., Lefèvre, A., Reynier, P., Eymieux, S., Blanchard, E., Vourc’h, P., et al. (2022) · Biomedicines · doi:10.3390/biomedicines10061307 · Cited by 24
Dib, M. (2003) · Drugs · doi:10.2165/00003495-200363030-00004 · Cited by 24
Candelise, N., Salvatori, I., Scaricamazza, S., Nesci, V., Zenuni, H., Ferri, A., & Valle, C. (2022) · Metabolites · doi:10.3390/metabo12030233 · Cited by 25
Manzano, R., Toivonen, J. M., Calvo, A. C., Oliván, S., Zaragoza, P., Muñoz, M. J., Montarras, D., & Osta, R. (2011) · Stem Cell Reviews and Reports · doi:10.1007/s12015-011-9268-0 · Cited by 23
Perry, J. J. P., Shin, D. S., & Tainer, J. A. (2010) · Advances in experimental medicine and biology · doi:10.1007/978-1-4419-6448-9_2 · Cited by 23
Limited diagnostic value of enzyme analysis in patients with mitochondrial tRNA mutations
Wibrand, F., Jeppesen, T. D., Frederiksen, A. L., Olsen, D. B., Dunø, M., Schwartz, M., & Vissing, J. (2009) · Muscle & Nerve · doi:10.1002/mus.21541 · Cited by 22
Yusoff, A. A. M., & Khair, S. Z. N. M. (2024) · Reviews in the Neurosciences · doi:10.1515/revneuro-2024-0080 · Cited by 23
Polyamine metabolism dysregulation contributes to muscle fiber vulnerability in ALS
Ruggieri, V., Scaricamazza, S., Bracaglia, A., D’Ercole, C., Parisi, C., D’Angelo, P., Proietti, D., Cappelletti, C., Macone, A., Lozanoska‐Ochser, B., et al. (2024) · Cell Reports · doi:10.1016/j.celrep.2024.115123 · Cited by 22
Sigma-1 Receptor in Motoneuron Disease
Mancuso, R., & Navarro, X. (2017) · Advances in experimental medicine and biology · doi:10.1007/978-3-319-50174-1_16 · Cited by 22
Lack of Effect of Methylene Blue in the SOD1 G93A Mouse Model of Amyotrophic Lateral Sclerosis
Lougheed, R., & Turnbull, J. (2011) · PLoS ONE · doi:10.1371/journal.pone.0023141 · Cited by 20
Amyotrophic lateral sclerosis: contemporary concepts in etiopathogenesis and pharmacotherapy
Strong, M. J. (2004) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.13.12.1593 · Cited by 20
Is ALS a systemic disorder? Evidence from muscle mitochondria
Appel, S. H. (2006) · Experimental Neurology · doi:10.1016/j.expneurol.2005.12.025 · Cited by 20
MITOCHONDRIOPATHY MIMICKING AMYOTROPHIC LATERAL SCLEROSIS
Finsterer, J. (2003) · The Neurologist · doi:10.1097/01.nrl.0000038589.58012.a8 · Cited by 20
Creatine and coenzyme Q10 in the treatment of ALS
Strong, M. J., & Pattee, G. L. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820050515665 · Cited by 20
Santa-Cruz, L. D., Guerrero‐Castillo, S., Uribe‐Carvajal, S., & Tapia, R. (2016) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.6b00032 · Cited by 19
Reduced exercise capacity in early-stage amyotrophic lateral sclerosis: Role of skeletal muscle
Mezzani, A., Pisano, F., Cavalli, A., Tommasi, M. A., Corrà, U., Colombo, S., Grassi, B., Marzorati, M., Porcelli, S., Morandi, L. O., et al. (2011) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482968.2011.601463 · Cited by 19
Mitochondriopathy as a differential diagnosis of amyotrophic lateral sclerosis
Finsterer, J. (2002) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608202760839008 · Cited by 19
Inflammatory cytokines increase mitochondrial damage in motoneuronal cells expressing mutant SOD1
Ferri, A., Nencini, M., Cozzolino, M., Carrara, P., Moreno, S., & Carrı̀, M. T. (2008) · Neurobiology of Disease · doi:10.1016/j.nbd.2008.08.004 · Cited by 17
Neuroprotective Effect of Human Adipose Stem Cell-Derived Extract in Amyotrophic Lateral Sclerosis
Jeon, G. S., Im, W., Shim, Y., Lee, M., Kim, M.-J., Hong, Y., Seong, S., Kim, M., & Sung, J. (2015) · Neurochemical Research · doi:10.1007/s11064-015-1774-z · Cited by 16
Mitochondrial genome aberrations in skeletal muscle of patients with motor neuron disease
Artuso, L., Zoccolella, S., Favia, P., Amati, A., Capozzo, R., Logroscino, G., Serlenga, L., Simone, I. L., Gasparre, G., & Petruzzella, V. (2012) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2012.735239 · Cited by 16
Rodriguez, G. E., González, D. M. C., Gargiulo-Monachelli, G. M., Costa, J. J. L., Nicola, A. F. D., & Sica, R. E. P. (2012) · Arquivos de Neuro-Psiquiatria · doi:10.1590/s0004-282x2012000100010 · Cited by 16
Mitochondrial disfunction as a cause of ALS.
Pizzuti, A., & Petrucci, S. (2011) · PubMed · doi:10.4449/aib.v149i1.1266 · Cited by 15
Multidisciplinary Interventions in Motor Neuron Disease
Williams, U. E., Philip-Ephraim, E. E., & Oparah, S. K. (2014) · Journal of Neurodegenerative Diseases · doi:10.1155/2014/435164 · Cited by 14
Amyotrophic Lateral Sclerosis and Metabolomics: Clinical Implication and Therapeutic Approach
Kumar, A., Ghosh, D., & Singh, R. L. (2013) · Journal of Biomarkers · doi:10.1155/2013/538765 · Cited by 15
Bradley, L. J., Taanman, J., Kallis, C., & Orrell, R. W. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.04.007 · Cited by 14
Eosinophilic Enteritis in Horses with Motor Neuron Disease
de Castro, E. D., Zafra, R., Acevedo, L. M., Pérez, J., Acosta, I., Rivero, J. L. L., & Aguilera‐Tejero, E. (2016) · Journal of Veterinary Internal Medicine · doi:10.1111/jvim.13944 · Cited by 14
The Cellular and Molecular Signature of ALS in Muscle
Anakor, E., Duddy, W. J., & Duguez, S. (2022) · Journal of Personalized Medicine · doi:10.3390/jpm12111868 · Cited by 14
Parvanovova, P., Evinová, A., Grofik, M., Hnilicová, P., Tatarková, Z., & Koprušáková, M. T. (2024) · Biomedicines · doi:10.3390/biomedicines12061294 · Cited by 14
Webber, D. S., Lopez, I., Korsak, R. A., Hirota, S. K., Acuña, D., & Edmond, J. (2005) · Journal of Neuroscience Research · doi:10.1002/jnr.20495 · Cited by 13
Shrivastava, M., & Vivekanandhan, S. (2011) · Ultrastructural Pathology · doi:10.3109/01913123.2011.553350 · Cited by 13
Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis and Spinal Muscular Atrophy
Strong, M. J., Hortobágyi, T., Okamoto, K., & Kato, S. (2011) · Wiley eBooks · doi:10.1002/9781444341256.ch44 · Cited by 13
Mathur, D., Riffo‐Campos, Á. L., Castillo, J., Haines, J. D., Vidaurre, Ó. G., Zhang, F., Ferrer, F. C., Casaccia, P., Casanova, B., & López‐Rodas, G. (2017) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2017.00209 · Cited by 12
Sica, R. E. P., Nicola, A. F. D., Deniselle, M. C. G., Rodriguez, G. E., Gargiulo-Monachelli, G. M., Peralta, L. M., & Bettini, M. (2011) · Arquivos de Neuro-Psiquiatria · doi:10.1590/s0004-282x2011000500023 · Cited by 10
Moreno‐Ortega, A. J., Al-achbili, L. M., Alonso, E., Rı́os, C. D. L., Garcı́a, A. G., Ruíz‐Nuño, A., & Cano‐Abad, M. F. (2016) · Neurotoxicity Research · doi:10.1007/s12640-016-9623-7 · Cited by 11
Tungtur, S. K., Wilkins, H., Rogers, R. S., Badawi, Y., Sage, J. M., Aǵbaş, A., Jawdat, O., Barohn, R. J., Swerdlow, R. H., & Nishimune, H. (2021) · Scientific Reports · doi:10.1038/s41598-021-90438-6 · Cited by 11
Mitochondria and amyotrophic lateral sclerosis
Orrell, R. W., & Schapira, A. H. V. (2002) · International review of neurobiology · doi:10.1016/s0074-7742(02)53015-4 · Cited by 11
Altered calcium homeostasis in ALS as a target for therapy
Appel, S. H. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820052415754-1 · Cited by 11
Varghese, M., Zhao, W., Yemul, S., Vempati, P., Radu, A., Simkovic, S., & Pasinetti, G. M. (2020) · Biomolecules · doi:10.3390/biom10050760 · Cited by 10
Targeting mitochondrial Ca2+ uptake for the treatment of amyotrophic lateral sclerosis
Zhong, R., Rua, M. T., & Wei‐LaPierre, L. (2023) · The Journal of Physiology · doi:10.1113/jp284143 · Cited by 12
Metadata
- Authors
- Wiedemann, F. R., Winkler, K., Kuznetsov, A. V., Bartels, C., Vielhaber, S., Feistner, H., & Kunz, W. S.
- Affiliations
- Klinikum Magdeburg (DE)
- University Hospital Magdeburg (DE)
- Corresponding author
- Kunz, Wolfram S
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1998-03
- Volume
- 156
- Issue
- 1
- Pages
- 65-72
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2020074935
- Cited by
- 262
- References
- 27
- Citations by year
- 2026: 9 · 2025: 7 · 2024: 7 · 2023: 5 · 2022: 10 · 2021: 9 · 2020: 9 · 2019: 6 · 2018: 6 · 2017: 8 · 2016: 10 · 2015: 9 · 2014: 10 · 2013: 11 · 2012: 14
- FWCI
- 6.41
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Keywords
- amyotrophic lateral sclerosis, mitochondrial dysfunction, skeletal muscle, oxidative phosphorylation, electron transport inhibition, amytal, flux control coefficients, NAD(P)H autofluorescence, spinal muscular atrophy
- MeSH terms
- Adult, Aged, Amyotrophic Lateral Sclerosis, Electromyography, Electron Transport, Female, Humans, Male, Microscopy, Fluorescence, Middle Aged, Mitochondria, Muscle, NAD(P)H Dehydrogenase (Quinone), Muscle, Skeletal
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review