The DOI matches its Crossref record; 68 of the 68 references it cites were found in a registry or an index.
Microglial Activation Correlates with Disease Progression and Upper Motor Neuron Clinical Symptoms in Amyotrophic Lateral Sclerosis
Open accessAbstract
BACKGROUND/AIMS: We evaluated clinicopathological correlates of upper motor neuron (UMN) damage in amyotrophic lateral sclerosis (ALS), and analyzed if the presence of the C9ORF72 repeat expansion was associated with alterations in microglial inflammatory activity. METHODS: Microglial pathology was assessed by IHC with 2 different antibodies (CD68, Iba1), myelin loss by Kluver-Barrera staining and myelin basic protein (MBP) IHC, and axonal loss by neurofilament protein (TA51) IHC, performed on 59 autopsy cases of ALS including 9 cases with C9ORF72 repeat expansion. RESULTS: Microglial pathology as depicted by CD68 and Iba1 was significantly more extensive in the corticospinal tract (CST) of ALS cases with a rapid progression of disease. Cases with C9ORF72 repeat expansion showed more extensive microglial pathology in the medulla and motor cortex which persisted after adjusting for disease duration in a logistic regression model. Higher scores on the clinical UMN scale correlated with increasing microglial pathology in the cervical CST. TDP-43 pathology was more extensive in the motor cortex of cases with rapid progression of disease. CONCLUSIONS: This study demonstrates that microglial pathology in the CST of ALS correlates with disease progression and is linked to severity of UMN deficits.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
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?
The cited source says what the claim attributes to it.
In the citing paper
“Neuroinflammation has also been observed in patients with ALS, in which inflammatory microglial hyperactivity in these patients has been linked to disease progression [50,54].”
From Head Injury and Amyotrophic Lateral Sclerosis: Population-Based Study from the National ALS Registry (2025) · cites this paper as [54]
In the citing paper
“ALS patients carrying C9orf72 mutations display microglial activation that correlates with disease progression [438].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [438]
In the citing paper
“Postmortem studies308–310 and in vivo PET studies311–313 revealed widespread cerebral microglial activation in patients with ALS,308–312 and even in pre-symptomatic ALS-related mutation carriers.309,313”
From Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023) · cites this paper as [309]
In the citing paper
“Microglial activation in the corticospinal tract of ALS correlated with neuronal and axonal loss and more severe upper motor neuron symptoms.309”
From Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023) · cites this paper as [309]
In the citing paper
“Transcriptome and histologic analyses of postmortem tissue from patients with ALS carrying the C9orf72 expansion (C9-ALS) found that decreased C9orf72 expression in C9-ALS led to altered microglial function and increased microglial activation.309,361”
From Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets (2023) · cites this paper as [309]
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
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
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
Self-citationInterrater Reliability of a Modified Ashworth Scale of Muscle Spasticity
Bohannon, R. W., & Smith, M. B. (1987) · Physical Therapy · doi:10.1093/ptj/67.2.206 · Cited by 5,461
El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis
Brooks, B. R., Miller, R. G., Swash, M., & Munsat, T. L. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608200300079536 · Cited by 5,433
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
Cedarbaum, J. M., Stambler, N., Malta, E., Fuller, C., Hilt, D., Thurmond, B., & Nakanishi, A. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00210-5 · Cited by 3,307
Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., Burrell, J. R., & Zoing, M. C. (2011) · The Lancet · doi:10.1016/s0140-6736(10)61156-7 · Cited by 2,603
Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia
Boillée, S., Yamanaka, K., Lobsiger, C. S., Copeland, N. G., Jenkins, N. A., Kassiotis, G., Kollias, G., & Cleveland, D. W. (2006) · Science · doi:10.1126/science.1123511 · Cited by 1,623
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
Flagged first, then by citations
Cited by
Sorted by citations
Items 101–200 of 268
UNC13A confers risk for sporadic ALS and influences survival in a Spanish cohort
Vidal-Taboada, J. M., López-López, A., Salvadó, M., Lorenzo-Sanz, L., García, C. B., Mahy, N., Rodríguez, M. J., & Gámez, J. (2015) · Journal of Neurology · doi:10.1007/s00415-015-7843-z · Cited by 40
Rojas, P., de Hoz, R., Ramirez, A. I., Ferreras, A., Salobrar‐García, E., Muñoz-Blanco, J. L., Urcelay-Segura, J. L., Salazar, J. J., & Ramirez, J. M. (2019) · Brain Sciences · doi:10.3390/brainsci9120337 · Cited by 40
Pagliardini, V., Pagliardini, S., Corrado, L., Lucenti, A., Panigati, L., Bersano, E., Servo, S., Cantello, R., D'Alfonso, S., & Mazzini, L. (2015) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2014.12.016 · Cited by 42
Ibudilast (MN-166) in amyotrophic lateral sclerosis- an open label, safety and pharmacodynamic trial
Babu, S., Hightower, B. G., Chan, J. G. M., Zürcher, N. R., Kivisäkk, P., Tseng, C.-E. J., Sanders, D. L., Robichaud, A., Banno, H., Evora, A., et al. (2021) · NeuroImage Clinical · doi:10.1016/j.nicl.2021.102672 · Cited by 39
Tzeplaeff, L., Jürs, A. V., Wohnrade, C., & Demleitner, A. F. (2024) · Cells · doi:10.3390/cells13050452 · Cited by 39
Yeo, H.-G., Hong, J. J., Lee, Y., Yi, K. S., Jeon, C., Park, J., Won, J., Seo, J., Ahn, Y., Kim, K., et al. (2019) · Experimental Neurobiology · doi:10.5607/en.2019.28.4.458 · Cited by 38
Ohgomori, T., Yamada, J., Takeuchi, H., Kadomatsu, K., & Jinno, S. (2016) · European Journal of Neuroscience · doi:10.1111/ejn.13227 · Cited by 38
Imaging of glia activation in people with primary lateral sclerosis
Paganoni, S., Alshikho, M. J., Zürcher, N. R., Cernasov, P., Babu, S., Loggia, M. L., Chan, J. G. M., Chonde, D. B., Izquierdo‐Garcia, D., Catana, C., et al. (2017) · NeuroImage Clinical · doi:10.1016/j.nicl.2017.10.024 · Cited by 36
Granucci, E. J., Griciuc, A., Mueller, K. A., Mills, A. N., Le, H., Dios, A. M., McGinty, D., Pereira, J. D., Elmaleh, D. R., Berry, J. D., et al. (2019) · Scientific Reports · doi:10.1038/s41598-019-53982-w · Cited by 37
A role for microglia in mediating the microbiota–gut–brain axis
Keane, L., Clarke, G. A., & Cryan, J. F. (2025) · Nature reviews. Immunology · doi:10.1038/s41577-025-01188-9 · Cited by 43
Physical exercise regulates microglia in health and disease
Strohm, A. O., & Majewska, A. K. (2024) · Frontiers in Neuroscience · doi:10.3389/fnins.2024.1420322 · Cited by 37
Gut microbiota immune cross-talk in amyotrophic lateral sclerosis
Kaul, M., Mukherjee, D., Weiner, H. L., & Cox, L. M. (2024) · Neurotherapeutics · doi:10.1016/j.neurot.2024.e00469 · Cited by 36
Fucich, E. A., Mayeux, J. P., McGinn, M. A., Gilpin, N. W., Edwards, S., & Molina, P. E. (2019) · Journal of Neurotrauma · doi:10.1089/neu.2018.5854 · Cited by 35
The role of immune-mediated alterations and disorders in ALS disease
Rodrigues Lima-Junior, J., Sulzer, D., Lindestam Arlehamn, C. S., & Sette, A. (2021) · Human Immunology · doi:10.1016/j.humimm.2021.01.017 · Cited by 33
C9ORF72 Mutations in Neurodegenerative Diseases
Liu, Y., Yu, J., Zong, Y., Zhou, J., & Tan, L. (2013) · Molecular Neurobiology · doi:10.1007/s12035-013-8528-1 · Cited by 36
Trageser, K. J., Smith, C., Herman, F. J., Ono, K., & Pasinetti, G. M. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.01298 · Cited by 31
Koza, L. A., Winter, A. N., Holsopple, J., Baybayon-Grandgeorge, A. N., Peña, C., Olson, J. R., Mazzarino, R. C., Patterson, D., & Linseman, D. A. (2020) · Nutrients · doi:10.3390/nu12061824 · Cited by 33
The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration
Ferreira, P. A. (2019) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-019-03029-0 · Cited by 34
Efficacy of Ciprofloxacin/Celecoxib combination in zebrafish models of amyotrophic lateral sclerosis
Goldshtein, H., Muhire, A., Légaré, V. P., Pushett, A., Rotkopf, R., Shefner, J. M., Peterson, R. T., Armstrong, G. A. B., & Russek‐Blum, N. (2020) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.51174 · Cited by 30
Glial Cell Dysfunction in C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Ghasemi, M., Keyhanian, K., & Douthwright, C. L. (2021) · Cells · doi:10.3390/cells10020249 · Cited by 35
New insights on the disease contribution of neuroinflammation in amyotrophic lateral sclerosis
Chiot, A., Lobsiger, C. S., & Boillée, S. (2019) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000729 · Cited by 34
Kondo, T., Banno, H., Okunomiya, T., Amino, Y., Endo, K., Nakakura, A., Uozumi, R., Kinoshita, A., Tada, H., Morita, S., et al. (2021) · BMJ Open · doi:10.1136/bmjopen-2021-051343 · Cited by 29
Urinary neopterin: A novel biomarker of disease progression in amyotrophic lateral sclerosis
Shepheard, S. R., Karnaros, V., Benyamin, B., Schultz, D. W., Fowler, M., Wuu, J., Chataway, T., Malaspina, A., Benatar, M. G., & Rogers, M. (2021) · European Journal of Neurology · doi:10.1111/ene.15237 · Cited by 29
Microglia in ALS: Insights into Mechanisms and Therapeutic Potential
Bond, S., Saxena, S., & Sierra-Delgado, J. A. (2025) · Cells · doi:10.3390/cells14060421 · Cited by 32
Fucich, E. A., Stielper, Z. F., Cancienne, H. L., Edwards, S., Gilpin, N. W., Molina, P. E., & Middleton, J. W. (2020) · Journal of Neurophysiology · doi:10.1152/jn.00570.2019 · Cited by 28
Neuroinflammation and Neurodegeneration
Morales, I., Farias, G. A., Cortés, N., & Maccioni, R. B. (2016) · InTech eBooks · doi:10.5772/64545 · Cited by 34
Recent advances in understanding amyotrophic lateral sclerosis and emerging therapies
Gittings, L. M., & Sattler, R. (2020) · Faculty Reviews · doi:10.12703/b/9-12 · Cited by 27
Shen, D., Hou, B., Xu, Y., Cui, B., Peng, P., Li, X., Tai, H., Zhang, K., Liu, S., Fu, H., et al. (2018) · Frontiers in Neurology · doi:10.3389/fneur.2018.00364 · Cited by 26
Review: Microglia in motor neuron disease
Ashford, B., Boche, D., Cooper‐Knock, J., Heath, P. R., Simpson, J. E., & Highley, J. R. (2020) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12640 · Cited by 27
Chitotriosidase as biomarker for early stage amyotrophic lateral sclerosis: a multicenter study
Steinacker, P., Feneberg, E., Halbgebauer, S., Witzel, S., Verde, F., Oeckl, P., Damme, P. V., Gaur, N., Gray, E., Großkreutz, J., et al. (2021) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1861023 · Cited by 27
Silva, K., Hope-Lucas, C., White, T., Hairston, T. K., Rameau, T., & Brown, A. M. (2015) · Journal of NeuroVirology · doi:10.1007/s13365-015-0317-3 · Cited by 25
The clinical and pathological phenotypes of frontotemporal dementia with C9ORF72 mutations
Liu, Y., Yu, J., Sun, F.-R., Ou, J.-R., Qu, S.-B., & Tan, L. (2013) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2013.09.013 · Cited by 27
Lorenzini, I., Alsop, E. B., Levy, J., Gittings, L. M., Lall, D., Rabichow, B. E., Moore, S. R., Pevey, R. S., Bustos, L. M., Burciu, C., et al. (2023) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1179796 · Cited by 34
Yang, Y., Rowe, D. B., McCann, H., Shepherd, C. E., Kril, J. J., Kiernan, M. C., Halliday, G. M., & Tan, R. H. (2023) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12919 · Cited by 23
A pilot study assessing T1-weighted muscle MRI in amyotrophic lateral sclerosis (ALS)
Diamanti, L., Alfonsi, E., Ferraro, O. E., Cereda, C., Pansarasa, O., Bastianello, S., & Pichiecchio, A. (2018) · Skeletal Radiology · doi:10.1007/s00256-018-3073-7 · Cited by 22
Vocht, J. D., Weehaeghe, D. V., Ombelet, F., Masrori, P., Lamaire, N., Devrome, M., Esch, H. V., Moisse, M., Koole, M. J. B., Dupont, P. J., et al. (2023) · Cells · doi:10.3390/cells12060933 · Cited by 22
Schreiber, S., Dębska–Vielhaber, G., Abdulla, S., Machts, J., Schreiber, F., Kropf, S., Körtvélyessy, P., Körner, S., Kollewe, K., Petri, S., et al. (2017) · Muscle & Nerve · doi:10.1002/mus.25682 · Cited by 22
Contributions of Drug Transporters to Blood-Brain Barriers
Liu, L., & Liu, X. (2019) · Advances in experimental medicine and biology · doi:10.1007/978-981-13-7647-4_9 · Cited by 21
Rifai, O. M., Longden, J., O’Shaughnessy, J., Sewell, M. D. E., Pate, J. A., McDade, K., Daniels, M. J. D., Abrahams, S., Chandran, S., McColl, B. W., et al. (2022) · The Journal of Pathology · doi:10.1002/path.6008 · Cited by 20
Microglia in brain aging: An overview of recent basic science and clinical research developments
Fan, H., Zhang, M., Wen, J., Wang, S., Yuan, M., Sun, H., Shu, L., Xu, Y., Pu, Y., & Cai, Z. (2023) · Journal of Biomedical Research · doi:10.7555/jbr.37.20220220 · Cited by 23
Pathogenic or protective? Neuropeptide Y in amyotrophic lateral sclerosis
Clark, C. M., Clark, R. M., Hoyle, J. A., & Dickson, T. C. (2020) · Journal of Neurochemistry · doi:10.1111/jnc.15125 · Cited by 20
Ashok, C., Kimble, A. L., Burrage, E. N., Helming, S. B., Tyburski, J. D., Leclair, N. K., Omar, O. M., Zuberi, A. R., Murphy, M., Jellison, E. R., et al. (2025) · Science Advances · doi:10.1126/sciadv.ads0505 · Cited by 21
Microglia Morphological Changes in the Motor Cortex of hSOD1G93A Transgenic ALS Mice
Migliarini, S., Scaricamazza, S., Valle, C., Ferri, A., Pasqualetti, M., & Ferraro, E. (2021) · Brain Sciences · doi:10.3390/brainsci11060807 · Cited by 21
Rifai, O. M., O’Shaughnessy, J., Dando, O., Munro, A. F., Sewell, M. D. E., Abrahams, S., Waldron, F. M., Sibley, C. R., & Gregory, J. M. (2023) · Brain · doi:10.1093/brain/awad243 · Cited by 23
L-type Calcium Channel Cav1.2 Is Required for Maintenance of Auditory Brainstem Nuclei
Ebbers, L., Satheesh, S. V., Janz, K., Rüttiger, L., Blosa, M., Hofmann, F. B., Morawski, M., Griesemer, D., Knipper, M., Friauf, E., et al. (2015) · Journal of Biological Chemistry · doi:10.1074/jbc.m115.672675 · Cited by 21
Neuropathological profile of long‐duration amyotrophic lateral sclerosis in military Veterans
Spencer, K. R., Foster, Z. W., Rauf, N. A., Guilderson, L., Collins, D., Averill, J. G., Walker, S. E., Robey, I., Cherry, J. D., Alvarez, V. E., et al. (2020) · Brain Pathology · doi:10.1111/bpa.12876 · Cited by 20
Fuller, D. D., Trejo‐Lopez, J. A., Yachnis, A. T., Sunshine, M. D., Rana, S. D., Bindi, V. E., Byrne, B. J., & Smith, B. K. (2021) · Journal of Neurophysiology · doi:10.1152/jn.00190.2021 · Cited by 19
(2023) · Advances in magnetic resonance technology and applications · doi:10.1016/b978-0-323-91771-1.00016-2 · Cited by 19
Urinary neopterin, a new marker of the neuroinflammatory status in amyotrophic lateral sclerosis
Lunetta, C., Lizio, A., Gerardi, F., Tarlarini, C., Filippi, M., Riva, N., Tremolizzo, L., Diamanti, S., Dellanoce, C., Mosca, L., et al. (2020) · Journal of Neurology · doi:10.1007/s00415-020-10047-7 · Cited by 19
Henderson, R. D., Agosti, J. M., McCombe, P., Thorpe, K., Heggie, S., Heshmat, S., Appleby, M. W., Ziegelaar, B. W., Crowe, D., & Redlich, G. L. (2021) · Medicine · doi:10.1097/md.0000000000027421 · Cited by 18
Immunological drivers of amyotrophic lateral sclerosis
Gendron, T. F., & Petrucelli, L. (2023) · Science Translational Medicine · doi:10.1126/scitranslmed.adj9332 · Cited by 18
Dhankhar, J., Agrawal, N., & Shrivastava, A. (2020) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2020.577302 · Cited by 17
Extracellular vesicles in TDP-43 proteinopathies: pathogenesis and biomarker potential
Dellar, E. R., Nikel, L., Fowler, S. L., Vahsen, B. F., Dafinca, R., Feneberg, E., Talbot, K., Turner, M. R., & Thompson, A. G. (2025) · Molecular Neurodegeneration · doi:10.1186/s13024-025-00859-4 · Cited by 17
Song, F., Chiang, P., Ravits, J., & Loeb, J. A. (2013) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2013.853802 · Cited by 17
Reyes‐Leiva, D., Dols‐Icardo, O., Sirisi, S., Cortés‐Vicente, E., Turón‐Sans, J., de Luna, N., Blesa, R., Belbin, O., Montal, V., Alcolea, D. A., et al. (2022) · Frontiers in Neurology · doi:10.3389/fneur.2021.750543 · Cited by 18
Clénet, M., Keaney, J., Gillet, G., Valadas, J. S., Langlois, J., Cardenas, A., Gasser, J., & Kadiu, I. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1190219 · Cited by 17
Toedebusch, C. M., Snyder, J. C., Jones, M. R., Garcia, V. B., Johnson, G. C., Villalón, E., Coates, J. R., & Garcia, M. L. (2018) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2018.01.009 · Cited by 16
Downer, O. M., Marcus, R. E. G., Zürcher, N. R., & Hooker, J. M. (2020) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.0c00362 · Cited by 15
Tennakoon, A., Katharesan, V., Musgrave, I., Koblar, S. A., Faull, R. L. M., Curtis, M. A., & Johnson, I. (2021) · Journal of Neuroscience Research · doi:10.1002/jnr.24996 · Cited by 17
Hayashi, S., Amari, M., & Okamoto, K. (2013) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2013.05.008 · Cited by 15
Krishnan, V. S., Shavlakadze, T., Grounds, M. D., Hodgetts, S. I., & Harvey, A. R. (2018) · Biogerontology · doi:10.1007/s10522-018-9765-5 · Cited by 15
Tuddenham, J. F., Fujita, M., Lee, E., Nimmagadda, N., Khairallah, A., Harbison, C., Flowers, X. E., Coronas‐Samano, G., Maniatis, S., Daly, A. C., et al. (2025) · Acta Neuropathologica · doi:10.1007/s00401-025-02913-3 · Cited by 16
Dellar, E. R., Vendrell, I., Talbot, K., Kessler, B. M., Fischer, R., Turner, M. R., & Thompson, A. G. (2023) · Journal of Neurochemistry · doi:10.1111/jnc.16030 · Cited by 13
The Current Potential Pathogenesis of Amyotrophic Lateral Sclerosis
Jiang, S., & Xu, R. (2024) · Molecular Neurobiology · doi:10.1007/s12035-024-04269-3 · Cited by 16
Ashok, C., Kimble, A. L., Tyburski, J. D., Leclair, N. K., Zuberi, A. R., Murphy, M., Jellison, E. R., Reese, B., Hu, X., Lutz, C. M., et al. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.12.13.571184 · Cited by 13
Chiot, A., Roemer, S. F., Ryner, L. C., Bogachuk, A. P., Emberley, K., Brownell, D., Jimenez, G. A., Leviten, M. W., Woltjer, R. L., Dickson, D. W., et al. (2023) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2306731120 · Cited by 12
Kovacs, M., Trías, E., Varela, V., Ibarburu, S., Beckman, J. S., Moura, I. C., Hermine, O., King, P. H., Si, Y., Kwon, Y., et al. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20163880 · Cited by 14
Urinary biomarkers for amyotrophic lateral sclerosis: candidates, opportunities and considerations
Rogers, M., Schultz, D. W., Karnaros, V., & Shepheard, S. R. (2023) · Brain Communications · doi:10.1093/braincomms/fcad287 · Cited by 13
Houghton, O. H., Mizielinska, S., & Gómez‐Suaga, P. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.838402 · Cited by 12
Ambrożkiewicz, K. A., Kozłowska, U., Haesler, V., Barnea, E. R., Mueller, M., & Kurpisz, M. K. (2022) · Scientific Reports · doi:10.1038/s41598-022-08064-9 · Cited by 11
Human VCP mutant ALS/FTD microglia display immune and lysosomal phenotypes independently of GPNMB
Clarke, B. E., Ziff, O. J., Tyzack, G. E., Petrić, M., Wang, Y., Klein, P., Smith, C. A., Hall, C. A., Helmy, A. E., Howell, M., et al. (2024) · Molecular Neurodegeneration · doi:10.1186/s13024-024-00773-1 · Cited by 14
Ashford, B., Simpson, J. E., Dawson, C., Boche, D., Cooper‐Knock, J., Heath, P. R., Fillingham, D. J., Appleby‐Mallinder, C., Wei, W., Dunning, M., et al. (2025) · Brain Pathology · doi:10.1111/bpa.70019 · Cited by 13
Liddell, J. R., Hilton, J. B. W., & Crouch, P. J. (2023) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12938 · Cited by 11
Saji, R., Uchio, R., Fuwa, A., Okuda-Hanafusa, C., KAWASAKI, K., Muroyama, K., Murosaki, S., Yamamoto, Y., & Hirose, Y. (2023) · Bioscience of Microbiota Food and Health · doi:10.12938/bmfh.2022-071 · Cited by 10
Wang, Y., & Patani, R. (2020) · Expert Opinion on Therapeutic Targets · doi:10.1080/14728222.2020.1805734 · Cited by 10
Evaluating emerging drugs in phase II & III for the treatment of amyotrophic lateral sclerosis
Li, X., & Bedlack, R. (2024) · Expert Opinion on Emerging Drugs · doi:10.1080/14728214.2024.2333420 · Cited by 11
Relevance of raised cerebrospinal fluid monocyte levels in patients with frontotemporal dementia
Pawlowski, M., Lueg, G., Groß, C. C., Johnen, A., Krämer, J., Weckesser, M., Wiendl, H., Meuth, S. G., & Duning, T. (2017) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2017.09.032 · Cited by 12
Rumman, M., Pandey, S., Singh, B., Gupta, M., & Mahdi, A. A. (2022) · Metabolic Brain Disease · doi:10.1007/s11011-022-01039-9 · Cited by 9
Gatto, R. G., Weissmann, C., Amin, M., Finkielsztein, A., Sumagin, R., Mareci, T. H., Uchitel, O. D., & Magin, R. L. (2020) · Animal Models and Experimental Medicine · doi:10.1002/ame2.12112 · Cited by 9
Imaging immunological processes from blood to brain in amyotrophic lateral sclerosis
Amor, S., Nutma, E., Marzin, M. C., & Puentes, F. (2021) · Clinical & Experimental Immunology · doi:10.1111/cei.13660 · Cited by 9
Meyer, M., Lima, A. E., Deniselle, M. C. G., & Nicola, A. F. D. (2022) · Cellular and Molecular Neurobiology · doi:10.1007/s10571-022-01294-5 · Cited by 9
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
Measuring disease progression in primary lateral sclerosis
Gilmore, M., Elman, L. B., Babu, S., Andres, P. L., & Floeter, M. K. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1837179 · Cited by 7
Self-citationThompson, A. G., Gray, E., Charles, P. D., Hu, M. T. M., Talbot, K., Fischer, R., Kessler, B. M., & Turner, M. R. (2021) · Frontiers in Neuroscience · doi:10.3389/fnins.2021.642324 · Cited by 7
Pathophysiological Correlation between Cigarette Smoking and Amyotrophic Lateral Sclerosis
Menounos, S., Hansbro, P. M., Diwan, A. D., & Das, A. (2021) · NeuroSci · doi:10.3390/neurosci2020008 · Cited by 6
Current neuroprotective therapies and future prospects for motor neuron disease
Evans, L. J., O’Brien, D., & Shaw, P. J. (2024) · International review of neurobiology · doi:10.1016/bs.irn.2024.04.003 · Cited by 5
Ferritin is closely associated with microglia in amyotrophic lateral sclerosis
Gao, J., Okolo, O. B., Siedlak, S. L., Friedland, R. P., & Wang, X. (2024) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/nlae074 · Cited by 6
Respiratory pathology in the TDP-43 transgenic mouse model of amyotrophic lateral sclerosis
Biswas, D. D., Sethi, R., Woldeyohannes, Y., Scarrow, E. R., Haddad, L. E., Lee, J., & ElMallah, M. K. (2024) · Frontiers in Physiology · doi:10.3389/fphys.2024.1430875 · Cited by 6
Rostalski, H., Hietanen, T., Leskelä, S., Beháňová, A., Abdollahzadeh, A., Wittrahm, R., Mäkinen, P. M., Huber, N., Hoffmann, D. B., Solje, E., et al. (2020) · Frontiers in Neurology · doi:10.3389/fneur.2020.550140 · Cited by 5
Cellular and molecular phenotypes of C9orf72 ALS/FTD patient derived iPSC-microglia mono-culturesPreprint
Lorenzini, I., Alsop, E. B., Levy, J., Gittings, L. M., Lall, D., Rabichow, B. E., Moore, S. R., Pevey, R. S., Bustos, L. M., Burciu, C., et al. (2020) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2020.09.03.277459 · Cited by 6
Wang, H., Liu, Y.-T., Ren, Y., Guo, X., & Wang, Y. (2024) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2024.578290 · Cited by 5
Epperly, M. W., Fisher, R., Rigatti, L. H., Watkins, S. C., Zhang, X., HOU, W., Shields, D. S., Franicola, D., Bayır, H., Wang, H., et al. (2019) · In Vivo · doi:10.21873/invivo.11526 · Cited by 5
Estimating acceleration time point of respiratory decline in ALS patients: A novel metric
Sato, K., Iwata, A., Kurihara, M., Nagashima, Y., Mano, T., & Toda, T. (2019) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2019.05.031 · Cited by 5
ALSUntangled No. 19: Sodium chlorite
The ALSUntangled Group (2013) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2013.769718 · Cited by 5
Current nano/biotechnological approaches in amyotrophic lateral sclerosis
Choi, I., Lee, E. H., & Lee, L. P. (2013) · Biomedical Engineering Letters · doi:10.1007/s13534-013-0114-y · Cited by 5
Head Injury and Amyotrophic Lateral Sclerosis: Population-Based Study from the National ALS Registry
Raymond, J., Howard, I. M., Berry, J., Larson, T., Horton, D. K., & Mehta, P. (2025) · Brain Sciences · doi:10.3390/brainsci15020143 · Cited by 4
Emerging anti-inflammatory nanosystems targeted to the brain
Mahanta, A. K., Gothwal, A., Chaulagain, B., Muolokwu, C. E., Tagoe, B., Lamsal, B., Belin, M. A. F., & Singh, J. (2025) · Nanomedicine · doi:10.1080/17435889.2025.2552103 · Cited by 4
dock8 deficiency attenuates microglia colonization in early zebrafish larvae
Wu, L., Xue, R., Chen, J., & Xu, J. (2022) · Cell Death Discovery · doi:10.1038/s41420-022-01155-6 · Cited by 4
The role of glial cells in amyotrophic lateral sclerosis
Pandya, V. A., & Patani, R. (2024) · International review of neurobiology · doi:10.1016/bs.irn.2024.04.005 · Cited by 5
Maetani, Y., Kurashige, T., Tada, Y., Kume, K., Watanabe, T., Sotomaru, Y., Yamanaka, K., Maruyama, H., & Kawakami, H. (2025) · Neuroscience Research · doi:10.1016/j.neures.2025.03.005 · Cited by 4
Metadata
- Authors
- Brettschneider, J., Toledo, J. B., Van Deerlin, V. M., Elman, L., McCluskey, L., Lee, V. M.-Y., & Trojanowski, J. Q.
- Affiliations
- Universität Ulm (DE)
- University of Pennsylvania (US)
- Corresponding author
- Brettschneider, Johannes
- Venue
- PLoS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2012-06-14
- Volume
- 7
- Issue
- 6
- Pages
- e39216
- ISSN
- 1932-6203 (ISSN-L)
- PMC
- PMC3375234
- OpenAlex
- W1974485241
- Cited by
- 268
- References
- 68
- Citations by year
- 2026: 16 · 2025: 18 · 2024: 23 · 2023: 27 · 2022: 24 · 2021: 25 · 2020: 24 · 2019: 24 · 2018: 15 · 2017: 22 · 2016: 10 · 2015: 13 · 2014: 12 · 2013: 13 · 2012: 2
- FWCI
- 8.26
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- PLoS ONE (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Figshare (submittedVersion, cc-by)
- Funders
- Deutsche Forschungsgemeinschaft (AG10124)
- National Institutes of Health (AG 10 124, AG 17586)
- National Institute on Aging (AG033101, AG-32953, P30AG010124, P01AG017586, AG17586, AG-10124, K08AG033101, P01AG032953)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- amyotrophic lateral sclerosis, microglial activation, upper motor neuron lesion, corticospinal tract, C9orf72 repeat expansion, disease progression, TDP-43 pathology, demyelination, axonal loss, neuroinflammation, motor cortex, medulla
- MeSH terms
- Aged, Amyotrophic Lateral Sclerosis, Axons, Base Sequence, Female, Humans, Male, Middle Aged, Motor Neurons, Cohort Studies, Polymerase Chain Reaction, Open Reading Frames, Microglia, DNA Primers, Disease Progression
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