Cited by 616Amyotrophic Lateral Sclerosis ResearchHereditary Neurological DisordersAlzheimer's disease research and treatments

The DOI matches its Crossref record; 201 of the 202 references it cites were found in a registry or an index.

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

Open access

Ferraiuolo, Laura, Kirby, Janine, Grierson, Andrew J., Sendtner, Michael, Shaw, Pamela J.·2011·Nature Reviews Neurology

DOI 10.1038/nrneurol.2011.152

Open this paper in CoChat — free account

Abstract

Amyotrophic lateral sclerosis (ALS) is a genetically diverse disease. At least 15 ALS-associated gene loci have so far been identified, and the causative gene is known in approximately 30% of familial ALS cases. Less is known about the factors underlying the sporadic form of the disease. The molecular mechanisms of motor neuron degeneration are best understood in the subtype of disease caused by mutations in superoxide dismutase 1, with a current consensus that motor neuron injury is caused by a complex interplay between multiple pathogenic processes. A key recent finding is that mutated TAR DNA-binding protein 43 is a major constituent of the ubiquitinated protein inclusions in ALS, providing a possible link between the genetic mutation and the cellular pathology. New insights have also indicated the importance of dysregulated glial cell-motor neuron crosstalk, and have highlighted the vulnerability of the distal axonal compartment early in the disease course. In addition, recent studies have suggested that disordered RNA processing is likely to represent a major contributing factor to motor neuron disease. Ongoing research on the cellular pathways highlighted in this Review is predicted to open the door to new therapeutic interventions to slow disease progression in ALS.

Integrity signals

Registry

Crossref · 10.1038/nrneurol.2011.152

Cites

201 of 201 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

We couldn’t retrieve this paper’s full text, so its claims and self-check weren’t run. The registry and citation checks still apply.

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?

2 retracted

RETRACTED: Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
Atkin, J. D., Farg, M. A., Walker, A. K., McLean, C., Tomas, D., & Horne, M. K. (2008) · Neurobiology of Disease · doi:10.1016/j.nbd.2008.02.009 · Cited by 350
Retracted
Induction of the Unfolded Protein Response in Familial Amyotrophic Lateral Sclerosis and Association of Protein-disulfide Isomerase with Superoxide Dismutase 1
Atkin, J. D., Farg, M. A., Turner, B. J., Tomas, D., Lysaght, J. A., Nunan, J., Rembach, A., Nagley, P., Beart, P. M., Cheema, S. S., et al. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m603393200 · Cited by 289
Retracted
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
Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
DeJesus-Hernandez, M., Mackenzie, I. R., Boeve, B. F., Boxer, A. L., Baker, M., Rutherford, N. J., Nicholson, A. M., Finch, N. A., Flynn, H., Adamson, J., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.011 · Cited by 4,936
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Renton, A. E., Majounie, E., Waite, A., Simón-Sánchez, J., Rollinson, S., Gibbs, J. R., Schymick, J. C., Laaksovirta, H., van Swieten, J. C., Myllykangas, L., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.010 · Cited by 4,409
Self-citation
Identification and characterization of a spinal muscular atrophy-determining gene
Lefebvre, S., Bürglen, L., Reboullet, S., Clermont, O., Burlet, P., Viollet, L., Bénichou, B., Cruaud, C., Millasseau, P., Zeviani, M., et al. (1995) · Cell · doi:10.1016/0092-8674(95)90460-3 · Cited by 3,995
Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17
Hutton, M., Lendon, C. L., Rizzu, P., Baker, M., Froelich, S., Houlden, H., Pickering-Brown, S., Chakraverty, S., Isaacs, A., Grover, A., et al. (1998) · Nature · doi:10.1038/31508 · Cited by 3,498
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Vance, C. A., Rogelj, B., Hortobágyi, T., Vos, K. J. D., Nishimura, A. L., Sreedharan, J., Hu, X., Smith, B. N., Ruddy, D., Wright, P. D., et al. (2009) · Science · doi:10.1126/science.1165942 · Cited by 2,625

Show all 202 references

Research works printed but not linked: 2

Fitzmaurice, P. S. et al. Evidence for DNA damage in amyotrophic lateral sclerosis. Muscle Nerve 19, 797–798 (1996).
Highley, J. R. et al. TARDBP mutations, amyotrophic lateral sclerosis and alternative splicing in human fibroblasts. Brain Pathol. 20 (Suppl. 1), 32 (2010).

Sorted by citations

Items 401–500 of 616

Identification of Amyotrophic Lateral Sclerosis Based on Diffusion Tensor Imaging and Support Vector Machine
Chen, Q., Zhang, X., Huang, N., & Chen, H. (2020) · Frontiers in Neurology · doi:10.3389/fneur.2020.00275 · Cited by 14
Mechanistic insights into the loss-of-function mechanisms of rare human D-amino acid oxidase variants implicated in amyotrophic lateral sclerosis
Padhi, A. K., & Zhang, K. Y. J. (2020) · Scientific Reports · doi:10.1038/s41598-020-74048-2 · Cited by 12
Perturbations of the Proteome and of Secreted Metabolites in Primary Astrocytes from the hSOD1(G93A) ALS Mouse Model
Stella, R., Bonadio, R. S., Cagnin, S., Massimino, M. L., Bertoli, A., & Peggion, C. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22137028 · Cited by 12
Amyotrophic lateral sclerosis models derived from human embryonic stem cells with different superoxide dismutase 1 mutations exhibit differential drug responses
Isobe, T., Tooi, N., Nakatsuji, N., & Aiba, K. (2015) · Stem Cell Research · doi:10.1016/j.scr.2015.09.006 · Cited by 12
A High-throughput and Pathophysiologically Relevant Astrocyte-motor Neuron Co-culture Assay for Amyotrophic Lateral Sclerosis Therapeutic Discovery
Stopford, M. J., Allen, S. P., & Ferraiuolo, L. (2019) · BIO-PROTOCOL · doi:10.21769/bioprotoc.3353 · Cited by 11
Self-citation
Bidens pilosa Extract Administered after Symptom Onset Attenuates Glial Activation, Improves Motor Performance, and Prolongs Survival in a Mouse Model of Amyotrophic Lateral Sclerosis
Kosuge, Y., Kaneko, E., Nango, H., Miyagishi, H., Ishige, K., & Ito, Y. (2020) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2020/1020673 · Cited by 12
Differential regulation of the glutamate transporter variants GLT-1a and GLT-1b in the cortex and spinal cord of transgenic rats expressing hSOD1G93A
Dumont, A. O., Hermans, E., & Goursaud, S. (2013) · Neurochemistry International · doi:10.1016/j.neuint.2013.04.012 · Cited by 12
An Exploratory Trial of EPI-589 in Amyotrophic Lateral Sclerosis (EPIC-ALS): Protocol for a Multicenter, Open-Labeled, 24-Week, Single-Group Study
Haji, S., Fujita, K., Oki, R., Osaki, Y., Miyamoto, R., Morino, H., Nagano, S., Atsuta, N., Kanazawa, Y., Matsumoto, Y., et al. (2022) · JMIR Research Protocols · doi:10.2196/42032 · Cited by 11
Motoneuron Disease
Sendtner, M. (2014) · Handbook of experimental pharmacology · doi:10.1007/978-3-642-45106-5_15 · Cited by 11
Self-citation
Dissecting the Effect of ALS Mutation G335D on the Early Aggregation of the TDP-43 Amyloidogenic Core Peptide: Helix-to-β-Sheet Transition and Conformational Shift
Li, F., Chen, Y., Tang, Y., Liu, X., & Wei, G. (2023) · Journal of Chemical Information and Modeling · doi:10.1021/acs.jcim.3c00513 · Cited by 11
Downstream Effects of Mutations in SOD1 and TARDBP Converge on Gene Expression Impairment in Patient-Derived Motor Neurons
Dash, B. P., Freischmidt, A., Weishaupt, J. H., & Hermann, A. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23179652 · Cited by 18
Amyotrophic Lateral Sclerosis as a Spatiotemporal Mislocalization Disease: Location, Location, Location
Gershoni‐Emek, N., Chein, M., Gluska, S., & Perlson, E. (2015) · International review of cytology · doi:10.1016/bs.ircmb.2014.11.003 · Cited by 11
CNTF, STAT3 and new therapies for axonal degeneration: what are they and what can they do?
Selvaraj, B. T., & Sendtner, M. (2013) · Expert Review of Neurotherapeutics · doi:10.1586/ern.13.9 · Cited by 11
Self-citation
Motor Neuron Disease: A Chemical Perspective
Wood, L. K., & Langford, S. J. (2014) · Journal of Medicinal Chemistry · doi:10.1021/jm5001584 · Cited by 11
Rare Angiogenin and Ribonuclease 4 variants associated with amyotrophic lateral sclerosis exhibit loss-of-function: a comprehensive in silico study
Padhi, A. K., Narain, P., & Gomes, J. (2019) · Metabolic Brain Disease · doi:10.1007/s11011-019-00473-6 · Cited by 11
Early alterations of RNA metabolism and splicing from adult corticospinal neurons in an ALS mouse modelPreprint
Marques, C., Fischer, M., Keime, C., Burg, T., Brunet, A., Scekic‐Zahirovic, J., & Rouaux, C. (2019) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/667733 · Cited by 11
Genetic Downregulation of the Metabotropic Glutamate Receptor Type 5 Dampens the Reactive and Neurotoxic Phenotype of Adult ALS Astrocytes
Torazza, C., Provenzano, F., Gallia, E., Cerminara, M., Balbi, M., Bonifacino, T., Tessitore, S., Ravera, S., Usai, C., Musante, I., et al. (2023) · Cells · doi:10.3390/cells12151952 · Cited by 11
Amyotrophic Lateral Sclerosis: Current Therapeutic Perspectives
Kumar, V., Kashav, T., & Hassan, M. I. (2018) · Springer eBooks · doi:10.1007/978-981-13-0944-1_19 · Cited by 10
Why do motor neurons degenerate? Actualisation in the pathogenesis of amyotrophic lateral sclerosis
Riancho, J., Gonzalo, I., Ruiz‐Soto, M., & Berciano, J. (2017) · Neurología (English Edition) · doi:10.1016/j.nrleng.2015.12.019 · Cited by 11
Botulinum Toxin Treatment of Adult Muscle Stem Cells from Children with Cerebral Palsy and hiPSC-Derived Neuromuscular Junctions
Costamagna, D., Bastianini, V., Corvelyn, M., Duelen, R., Deschrevel, J., Beukelaer, N. D., Houwer, H. D., Sampaolesi, M., Gayan‐Ramirez, G., Campenhout, A. V., et al. (2023) · Cells · doi:10.3390/cells12162072 · Cited by 9
Pathomechanistic Networks of Motor System Injury in Amyotrophic Lateral Sclerosis
Dey, B., Kumar, A., & Patel, A. B. (2023) · Current Neuropharmacology · doi:10.2174/1570159x21666230824091601 · Cited by 9
The 5’-Untranslated Region of the C9orf72 mRNA Exhibits a Phylogenetic Alignment to the Cis-Aconitase Iron-Responsive Element; Novel Therapies for Amytrophic Lateral Sclerosis
Lu, M., Rajanala, S., Mikkilineni, S., Cahill, C. M., Brown, R., Berry, J. D., & Rogers, J. T. (2016) · Neuroscience & Medicine · doi:10.4236/nm.2016.71003 · Cited by 9
Integrated phylogeny of the human brain and pathobiology of Alzheimer’s disease: A unifying hypothesis
Ovsepian, S. V., O’Leary, V. B., Höschl, C., & Záborszky, L. (2021) · Neuroscience Letters · doi:10.1016/j.neulet.2021.135895 · Cited by 9
Atomistic Insights into A315E Mutation-Enhanced Pathogenicity of TDP-43 Core Fibrils
Li, F., Chen, Y., Liu, X., Tang, Y., Dong, X., & Wei, G. (2022) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.2c00416 · Cited by 9
Biomarkers and molecular mechanisms of Amyotrophic Lateral Sclerosis
Chakraborty, A. K., & Diwan, A. R. (2022) · AIMS neuroscience · doi:10.3934/neuroscience.2022023 · Cited by 10
Positron emission tomography in amyotrophic lateral sclerosis: Towards targeting of molecular pathological hallmarks
Willekens, S. M. A., Weehaeghe, D. V., Damme, P. V., & Laere, K. V. (2016) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-016-3587-y · Cited by 9
SOD1-targeting therapies for neurodegenerative diseases: a review of current findings and future potential
Franklin, J., Azzouz, M., & Shaw, P. J. (2020) · Expert Opinion on Orphan Drugs · doi:10.1080/21678707.2020.1835638 · Cited by 8
Self-citation
Reversing Dysdynamism to Interrupt Mitochondrial Degeneration in Amyotrophic Lateral Sclerosis
Dorn, G. W. (2023) · Cells · doi:10.3390/cells12081188 · Cited by 8
Tetramethylpyrazine Nitrone in Amyotrophic Lateral Sclerosis
Liu, X., Shang, H., Wei, Q., Yao, X., Lian, L., Dang, J., Jia, R., Wu, Z., Li, H., Niu, Q., et al. (2025) · JAMA Network Open · doi:10.1001/jamanetworkopen.2024.61055 · Cited by 8
Amyotrophic Lateral Sclerosis
Lonergan, R., Mistumoto, H., & Murray, B. (2013) · Springer eBooks · doi:10.1007/978-1-4614-6567-6_20 · Cited by 8
Analyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets
Watts, M. E., Giadone, R. M., Ordureau, A., Holton, K. M., Harper, J. W., & Rubin, L. L. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1327361 · Cited by 7
Edaravone for the Treatment of Motor Neurone Disease: A Critical Review of Approved and Alternative Formulations against a Proposed Quality Target Product Profile
O’Neill, R., Yoo, O., Burcham, P. C., & Lim, L. Y. (2024) · Pharmaceutics · doi:10.3390/pharmaceutics16080993 · Cited by 7
Focus on the excitatory and inhibitory neurotransmission imbalance in amyotrophic lateral sclerosis: a harmful disease player or a potential therapeutic opportunity?
Tessitore, S., Torazza, C., Bonifacino, T., Bacchetti, F., Roselli, F., Raiteri, L., Milanese, M., & Bonanno, G. (2026) · Neurobiology of Disease · doi:10.1016/j.nbd.2026.107272 · Cited by 8
Potential Applications for Growth Hormone Secretagogues Treatment of Amyotrophic Lateral Sclerosis
Meanti, R., Bresciani, E., Rizzi, L., Coco, S., Zambelli, V., Dimitroulas, A., Molteni, L., Omeljaniuk, R. J., Locatelli, V., & Torsello, A. (2022) · Current Neuropharmacology · doi:10.2174/1570159x20666220915103613 · Cited by 7
Caenorhabditis elegans as a model system to evaluate neuroprotective potential of nano formulations
Chauhan, P., Wadhwa, K., & Singh, G. (2022) · Frontiers in Nanotechnology · doi:10.3389/fnano.2022.1018754 · Cited by 8
Personalized Treatment for Infantile Ascending Hereditary Spastic Paralysis Based on In Silico Strategies
Sebastiano, M. R., Ermondi, G., Sato, K., Otomo, A., Hadano, S., & Caron, G. (2022) · Molecules · doi:10.3390/molecules27207063 · Cited by 7
Amyotrophic Lateral Sclerosis-Associated Mutants of SOD1 Modulate miRNA Biogenesis through Aberrant Interactions with Exportin 5
Chen, X., He, X., Yang, Y., & Wang, Y. (2022) · ACS Chemical Biology · doi:10.1021/acschembio.2c00591 · Cited by 7
Sex-Specific Early Retinal Dysfunction in Mutant TDP-43 Transgenic Mice
Gao, J., Leinonen, H., Wang, E. J., Ding, M., Perry, G. L., Palczewski, K., & Wang, X. (2023) · Journal of Alzheimer s Disease · doi:10.3233/jad-231102 · Cited by 7
Mechanism and implications of traditional Chinese medicine in amyotrophic lateral sclerosis therapy
Kumar, V., Gupta, P., & Hassan, M. I. (2019) · Journal of Proteins and Proteomics · doi:10.1007/s42485-019-00009-7 · Cited by 7
Amyotrophic Lateral Sclerosis
Hernández, A. (2017) · Open access Journal of Neurology & Neurosurgery · doi:10.19080/oajnn.2017.03.555609 · Cited by 7
A(a)LS: Ammonia-induced amyotrophic lateral sclerosis
Parekh, B. K. (2015) · F1000Research · doi:10.12688/f1000research.6364.1 · Cited by 7
Targeting Amyotrophic Lateral Sclerosis with Gene Therapy: From Silencing Genes to Enhancing Neuroprotection
Verdés, S., Navarro, X., & Bosch, A. (2025) · Human Gene Therapy · doi:10.1177/10430342251372898 · Cited by 8
Overexpression of autophagy enhancer PACER/RUBCNL in neurons accelerates disease in the SOD1G93A ALS mouse model
Labrador, L., Rodriguez, L., Beltrán, S., Hernandez, F., Gómez, L., Ojeda, P., Bergmann, C. A., Nassif, M., Kerr, B., Medinas, D. B., et al. (2024) · Biological Research · doi:10.1186/s40659-024-00567-1 · Cited by 6
Emerging drug targets in amyotrophic lateral sclerosis
Bova, M. P., & Kinney, G. G. (2012) · Expert Opinion on Orphan Drugs · doi:10.1080/21678707.2013.744949 · Cited by 6
Enhanced Cortical Metabolic Activity in Females and Males of a Slow Progressing Mouse Model of Amyotrophic Lateral Sclerosis
Roy, D., Puvvada, M., Kapanaiah, S. K. T., & Patel, A. B. (2022) · Neurochemical Research · doi:10.1007/s11064-022-03568-2 · Cited by 6
Neuroprotective effects of lithium in neuropsychiatric disorders
Agam, G., & Levine, J. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-814037-6.00006-9 · Cited by 6
EPIGENETIC MECHANISMS IN ALZHEIMER'S DISEASE: STATE-OF-THE-ART
Faltraco, F., Lista, S., Garaci, F., & Hampel, H. (2012) · Cineca Institutional Research Information System (Tor Vergata University) · Cited by 5
Distribution Features and Potential Effects of Serotonin in the Cerebrum of SOD1 G93A Transgenic Mice
He, P., He, B., Li, S., Chai, W., Rao, W., Zhu, Y. T., Chen, W., Zhang, P., Zhang, X., Pan, H., et al. (2022) · eNeuro · doi:10.1523/eneuro.0001-22.2022 · Cited by 5
Persistent NRG1 Type III Overexpression in Spinal Motor Neurons Has No Therapeutic Effect on ALS-Related Pathology in SOD1G93A Mice
Hernández, S., Salvany, S., Casanovas, A., Piedrafita, L., Soto‐Bernardini, M. C., Tarabal, O., Blasco, A., Gras, S., Gatius, A., Schwab, M. H., et al. (2023) · Neurotherapeutics · doi:10.1007/s13311-023-01424-x · Cited by 5
Contractile characteristics of gastrocnemius-soleus muscle in the SOD1G93A ALS mouse model
Dibaj, P., Schomburg, E. D., & Steffens, H. (2015) · Neurological Research · doi:10.1179/1743132815y.0000000039 · Cited by 5
ANGDelMut – a web-based tool for predicting and analyzing functional loss mechanisms of amyotrophic lateral sclerosis-associated angiogenin mutations
Padhi, A. K., Vasaikar, S. V., Jayaram, B., & Gomes, J. (2014) · F1000Research · doi:10.12688/f1000research.2-227.v3 · Cited by 5
FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion
Fratta, P., Malik, B. A., Gray, A. L., Spada, A. R. L., Hanna, M. G., Fisher, E., & Greensmith, L. (2012) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2012.09.008 · Cited by 5
NMR Characterization of Angiogenin Variants and tRNAAla Products Impacting Aberrant Protein Oligomerization
Fagagnini, A., Fasoli, S., Garavís, M., Gómez‐Pinto, I., Gotte, G., & Laurents, D. V. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22031439 · Cited by 5
Altered transcriptome and disease-related phenotype emerge only after fibroblasts harvested from patients with age-related macular degeneration are differentiated into retinal pigment epithelium
Cai, H., Gong, J., Noggle, S., Paull, D. J., Rizzolo, L. J., Priore, L. V. D., & Fields, M. A. (2021) · Experimental Eye Research · doi:10.1016/j.exer.2021.108576 · Cited by 5
References
Shaw, C. A. (2017) · Wiley eBooks · doi:10.1002/9781118634523.refs · Cited by 9
Application of Neurotrophic Factors as a Therapeutic Approach for Neurodegenerative Diseases
Kim, S., Lee, S. Y., Jung, U. J., & Kim, S. R. (2025) · Experimental Neurobiology · doi:10.5607/en25023 · Cited by 5
A Simple and Efficient Method for Concomitant Isolation and Culture of Enriched Astroglial and Microglial Cells from the Rat Spinal Cord
Mishra, P. S., & Raju, T. R. (2020) · BIO-PROTOCOL · doi:10.21769/bioprotoc.3501 · Cited by 4
A combined clinical and computational approach to understand the SOD1A4T-mediated pathogenesis of rapidly progressive familial amyotrophic lateral sclerosis
Diker, S., Gelener, P., Teralı, K., Ergören, M. Ç., Tunca, C., Başak, A. N., & Tan, E. (2021) · Acta Neurologica Belgica · doi:10.1007/s13760-020-01588-9 · Cited by 4
Altered Immunomodulatory Responses in the CX3CL1/CX3CR1 Axis Mediated by hMSCs in an Early In Vitro SOD1G93A Model of ALS
Sarikidi, A., Kefalakes, E., Falk, C. S., Esser, R., Ganser, A., Thau‐Habermann, N., & Petri, S. (2022) · Biomedicines · doi:10.3390/biomedicines10112916 · Cited by 4
Rutin Ameliorates ALS Pathology by Reducing SOD1 Aggregation and Neuroinflammation in an SOD1-G93A Mouse Model
Du, X., Dong, Q.-X., Zhu, J., Li, L., Yu, X., & Liu, R. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms251910392 · Cited by 4
Preclinical evaluation of cannabidiolic acid as a neuroprotective agent in TDP-43 transgenic mice, an experimental model of amyotrophic lateral sclerosis
García‐Toscano, L., Rodríguez‐Cueto, C., Furiano, A., Hind, W. H., de Lago, E., & Fernández‐Ruíz, J. (2025) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2025.118288 · Cited by 4
Far beyond the motor neuron: the role of glial cells in amyotrophic lateral sclerosis
de Souza, P. V. S., Pinto, W. B. V. D. R., Filho, F. M. R., & Oliveira, A. S. B. (2016) · Arquivos de Neuro-Psiquiatria · doi:10.1590/0004-282x20160117 · Cited by 4
Autopsy‐proven case of paraneoplastic lower motor neuron disease with sensorimotor neuropathy due to Waldenström's macroglobulinemia
Suzuki, Y., Aizawa, H., Sakashita, K., Kishi, H., Nomura, K., Yoshida, K., Aburakawa, Y., Kuroda, K., Murakami, C., Kakinoki, Y., et al. (2018) · Neuropathology · doi:10.1111/neup.12506 · Cited by 4
Genetic induction of hypometabolism by ablation of MC4R does not suppress ALS-like phenotypes in the G93A mutant SOD1 mouse model
Doshi, S., Gupta, P., & Kalb, R. G. (2017) · Scientific Reports · doi:10.1038/s41598-017-13304-4 · Cited by 5
Molecular Mechanisms of Neurological Disease
Gireud, M., Sirisaengtaksin, N., & Bean, A. J. (2014) · Elsevier eBooks · doi:10.1016/b978-0-12-397179-1.00021-x · Cited by 5
P75 and phosphorylated c-Jun are differentially regulated in spinal motoneurons following axotomy in rats.
Yuan, Q., Su, H., Wu, W., & Lin, Z. (2012) · Europe PMC (PubMed Central) · doi:10.3969/j.issn.1673-5374.2012.26.001 · Cited by 4
Genetics of Amyotrophic Lateral Sclerosis
Gorodenker, J., & Levy, L. M. (2014) · American Journal of Neuroradiology · doi:10.3174/ajnr.a3831 · Cited by 4
Mechanizmy patogenetyczne stwardnienia bocznego zanikowego
Iłżecka, J. (2012) · DOAJ (DOAJ: Directory of Open Access Journals) · Cited by 4
Highly responsive single-fluorophore indicator to explore lactate dynamics in high calcium environmentsPreprint
Galaz, A., Sandoval, P. Y., Soto-Ojeda, I., Hertenstein, H., Schweizer, J., Schirmeier, S., Barros, L. F., & Martín, A. S. (2020) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2020.10.01.322404 · Cited by 4
A Neglected Gene: The Role of the ANG Gene in the Pathogenesis of Amyotrophic Lateral Sclerosis
Zhang, Y., Li, Y., Bin, S., Cheng, X., & Niu, Q. (2024) · Aging and Disease · doi:10.14336/ad.2024.0107 · Cited by 4
A Comprehensive Review of Current Assistive Technology Research for Paralyzed People
Memon, Q. A. (2024) · Studies in computational intelligence · doi:10.1007/978-3-031-61463-7_15 · Cited by 3
Neuroaxonal Degeneration as a Converging Mechanism in Motor Neuron Diseases (MNDs): Molecular Insights into RNA Dysregulation and Emerging Therapeutic Targets
Sharbafshaaer, M., Pepe, R., Notariale, R., Canale, F., Tessitore, A., Tedeschi, G., & Trojsi, F. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms26157644 · Cited by 4
Stem Cell Approaches for Treatment of Neurodegenerative Diseases
Anand, S. (2014) · Clinical Pharmacology & Biopharmaceutics · doi:10.4172/2167-065x.1000126 · Cited by 3
Neurotrophic Therapy for ALS/MND
Rogers, M. (2014) · Springer eBooks · doi:10.1007/978-1-4614-5836-4_34 · Cited by 3
Neurotrophic Properties of C-Terminal Domain of the Heavy Chain of Tetanus Toxin on Motor Neuron Disease
Herrando‐Grabulosa, M., Casas, C., Talbot, K., & Aguilera, J. (2020) · Toxins · doi:10.3390/toxins12100666 · Cited by 3
Add-on treatment with Cerebrolysin improves clinical symptoms in patients with ALS: results from a prospective, single-center, placebo-controlled, randomized, double-blind, phase II study
Firstenfeld, A. J., Servicio de Neurociencias, U. D. B. A., Listorti, J., Servicio de Neurociencias, U. D. B. A., Jalaff, N., Servicio de Neurociencias, U. D. B. A., Orozco, C., Servicio de Neurociencias, U. D. B. A., Gosdenovich, F. N., Servicio de Neurociencias, U. D. B. A., et al. (2023) · Journal of Medicine and Life · doi:10.25122/jml-2023-0459 · Cited by 4
DNA Methylation in Psychiatric Diseases
Neidhart, M. (2015) · Elsevier eBooks · doi:10.1016/b978-0-12-420194-1.00017-8 · Cited by 3
Nutritional Support in Amyotrophic Lateral Sclerosis
Stavroulakis, T., & McDermott, C. J. (2017) · Springer eBooks · doi:10.1007/978-3-319-53171-7_6 · Cited by 3
Neutrophil-Lymphocyte Ratio (NLR) and Platelet-Lymphocyte Ratio (PLR) as neuroinflammatory biomarkers in Amyotrophic Lateral Sclerosis (ALS) progression
Santos, K. D. F., Azevedo, R. M., da Costa, C. C. P., da Cruz, P. B. D., Reis, A. A. D. S., & Santos, R. D. S. (2022) · Research, Society and Development · doi:10.33448/rsd-v11i5.28073 · Cited by 3
Cyclo (His-Pro) Protects SOD1G93A Microglial Cells from Paraquat–Induced Toxicity
Bellezza, S. G. I. (2015) · Journal of Clinical & Cellular Immunology · doi:10.4172/2155-9899.1000287 · Cited by 3
Increasing Power by Sharing Information from Genetic Background and Treatment in Clustering of Gene Expression Time Series
AL-Rashid, S. Z., Rahman, M. A., Al-aaraji, N. H. K., Lawrence, N. D., & Heath, P. R. (2018) · Journal of University of Babylon for Pure and Applied Sciences · doi:10.29196/jub.v26i4.711 · Cited by 3
Nerve and Muscle Disease
Lunn, M. P., Hanna, M. G., Howard, R., Parton, M. J., Rahman, S., Reilly, M. M., Sidle, K. C. L., & Turner, C. (2016) · doi:10.1002/9781118486160.ch10 · Cited by 3
TSPO Expression and [18 F ] DPA ‐714 PET / CT Imaging as Pathogenetic and Diagnostic Biomarkers in Symptomatic Stages of Skeletal Muscle Fiber Degeneration in SOD 1‐ G 93 A ALS Mice
Anzilotti, S., Iesu, N. D., Gargiulo, S., Muraglia, N. D., Cicatiello, A. G., Dentice, M., Panico, M., Albanese, S., Annunziato, L., Salvatore, M., et al. (2025) · The FASEB Journal · doi:10.1096/fj.202502450r · Cited by 2
siRNA for REST ameliorates symptoms in ALS mice and serum REST predicts disease prognosis and survival in ALS patients
Guida, N., Valsecchi, V., Anzilotti, S., Dubbioso, R., Cuomo, O., Ruggiero, S., Senerchia, G., Iuzzolino, V. V., Kolici, X., Iesu, N. D., et al. (2025) · Molecular Therapy · doi:10.1016/j.ymthe.2025.10.039 · Cited by 2
Allicin improves motor neuron survival in amyotrophic lateral sclerosis by reducing neuroinflammation and modulating gut microbiota
Gao, Y., Lu, Y., Zhao, S., Chen, R., Liu, J., Zhang, S., Bai, X., & Zhang, J. (2026) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2026.153503 · Cited by 2
Transcriptomic Analysis Reveals the Beneficial Effects of Spermidine in an ALS Mouse Model
Fiorucci, C., Rossi, M. N., Santo, R. D., Salvatori, I., Scaricamazza, S., Giuliani, S., Carletta, O., Filomena, E., Laurenti, D., Mattioli, R., et al. (2026) · Biomolecules · doi:10.3390/biom16040566 · Cited by 2
The Use of Human Samples to Study Familial and Sporadic Amyotrophic Lateral Sclerosis: New Frontiers and Challenges
Ferraiuolo, L., Meyer, K., & Kaspar, B. K. (2013) · InTech eBooks · doi:10.5772/56487 · Cited by 3
Self-citation
Nutrition and neurodegenerative diseases: Alzheimer's disease and others
Bianchi, V. E. (2024) · Elsevier eBooks · doi:10.1016/b978-0-323-91747-6.00013-5 · Cited by 2
Analysis of translatomic changes in the Ubqln2P497S model of ALS reveals that motor neurons express muscle-associated genes in non-disease states
Stansberry, W. M., Fiur, N. C., Robins, M. M., & Pierchala, B. A. (2024) · Frontiers in Neurology · doi:10.3389/fneur.2024.1491415 · Cited by 2
Blood DNA Methylation in Nuclear and Mitochondrial Sequences Links to Malnutrition and Poor Prognosis in ALS: A Longitudinal Study
Fernández‐Pombo, A., Izquierdo, A. G., Cantón-Blanco, A., García‐Sobrino, T., Hervás, D., Olmos, M. Á. M., Pardo, J., & Crujeiras, A. B. (2025) · Nutrients · doi:10.3390/nu17081295 · Cited by 2
LETM1 and mitochondrial calcium homeostasis: A controversial but critical role in cellular function and disease
dos Santos, G. R. R. M., Queiroz, M. I. C., & Leite, A. C. R. (2025) · Cell Calcium · doi:10.1016/j.ceca.2025.103085 · Cited by 2
Dysregulated expression and distribution of Kif5α in neurites of wobbler motor neurons
Matschke, V., Kürten, K., Gude, A.-C., Epplen, A. S. C., Stein, J., & Theiß, C. (2022) · Neural Regeneration Research · doi:10.4103/1673-5374.343883 · Cited by 2
Lower motor neurons – Counting cogs in the ALS machine
Simon, N. G. (2016) · Clinical Neurophysiology · doi:10.1016/j.clinph.2016.03.023 · Cited by 2
Regulation of Synaptic Transmission by Adenosine at the Neuromuscular Junction
Pousinha, P. A., & Ribeiro, J. A. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-803724-9.00004-1 · Cited by 2
Sporadic amyotrophic lateral sclerosis with seropositive neuromyelitis optica spectrum disorder
Kim, J. Y., Oh, H. J., Kim, Y., & Seok, J. M. (2021) · Medicine · doi:10.1097/md.0000000000025580 · Cited by 2
Imaging Biomarkers in Amyotrophic Lateral Sclerosis
Alberich, L. C., Vazquez-Costa, J. F., Ten‐Esteve, A., Mazón, M., & Martí‐Bonmatí, L. (2021) · Neuromethods · doi:10.1007/978-1-0716-1712-0_20 · Cited by 2
Nanoceuticals as Theranostics Against Neurodegenerative Diseases
Mishra, P., & Anand, S. (2022) · Nutritional neurosciences · doi:10.1007/978-981-16-8158-5_14 · Cited by 2
ANGDelMut – a web-based tool for predicting and analyzing functional loss mechanisms of amyotrophic lateral sclerosis-associated angiogenin mutations
Padhi, A. K., Vasaikar, S. V., Jayaram, B., & Gomes, J. (2013) · F1000Research · doi:10.12688/f1000research.2-227.v2 · Cited by 2
Exploring motor neuron degeneration in ALS - prevention by glycoursodeoxycholic acid and signaling to microglia
Cunha, J. C. M. (2012) · Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT) · Cited by 2
Zebrafish C9orf72 loss-of-function models of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Rounding, N. (2018) · White Rose eTheses Online (University of Leeds, The University of Sheffield, University of York) · Cited by 2

Authors: Ferraiuolo, L., Kirby, J., Grierson, A. J., Sendtner, M., & Shaw, P. J.
Affiliations: University of Sheffield (GB)
University of Würzburg (DE)
Corresponding author: Shaw, Pamela J.
ORCID: Ferraiuolo, Laura
Kirby, Janine
Grierson, Andrew J.
Sendtner, Michael
Shaw, Pamela J.
Venue: Nature Reviews Neurology
Publisher: Springer Science and Business Media LLC
Published: 2011-11
Volume: 7
Issue: 11
Pages: 616-630
ISSN: 1759-4758 (ISSN-L)
1759-4766
DOI: 10.1038/nrneurol.2011.152
OpenAlex: W2025413109
Cited by: 615
References: 202
Citations by year: 2026: 12 · 2025: 24 · 2024: 34 · 2023: 25 · 2022: 27 · 2021: 54 · 2020: 34 · 2019: 37 · 2018: 33 · 2017: 42 · 2016: 58 · 2015: 63 · 2014: 63 · 2013: 64 · 2012: 45
FWCI: 6.83
Type: journal article
Language: en
Open access: green
zenodo.org
Licence: other-oa
www.springer.com/tdm (tdm)
Locations: Nature Reviews Neurology (publishedVersion)
PubMed (publishedVersion)
Zenodo (CERN European Organization for Nuclear Research) (submittedVersion, other-oa)
Funders: Wellcome Trust
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: amyotrophic lateral sclerosis, motor neuron injury, motor neuron degeneration, oxidative stress, mitochondrial dysfunction, excitotoxicity, superoxide dismutase 1, TDP-43, sporadic ALS, familial ALS, therapeutic target
MeSH terms: Amyotrophic Lateral Sclerosis, Humans, Motor Neurons, Nerve Degeneration

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

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Citations

Cites

Cited by

Related

Metadata

Record

About these checks