Cited by 175Amyotrophic Lateral Sclerosis ResearchNeuroinflammation and Neurodegeneration MechanismsMultiple Sclerosis Research Studies

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Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia

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Lall, Deepti, Baloh, Robert H.·2017·Journal of Clinical Investigation

DOI 10.1172/jci90607PMC PMC5669558

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Abstract

Amyotrophic lateral sclerosis (ALS) is a degenerative disorder that is characterized by loss of motor neurons and shows clinical, pathological, and genetic overlap with frontotemporal dementia (FTD). Activated microglia are a universal feature of ALS/FTD pathology; however, their role in disease pathogenesis remains incompletely understood. The recent discovery that ORF 72 on chromosome 9 (C9orf72), the gene most commonly mutated in ALS/FTD, has an important role in myeloid cells opened the possibility that altered microglial function plays an active role in disease. This Review highlights the contribution of microglia to ALS/FTD pathogenesis, discusses the connection between autoimmunity and ALS/FTD, and explores the possibility that C9orf72 and other ALS/FTD genes may have a "dual effect" on both neuronal and myeloid cell function that could explain a shared propensity for altered systemic immunity and neurodegeneration.

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Background
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“Under normal physiological conditions, microglia typically eliminate TDP-43 via phagocytosis, whereas in its absence, a distinctive sequence of events that involves TDP-43 nucleocytoplasmic redistribution and neurodegeneration occurs, as observed in vivo using zebrafish spinal cord imaging [109].”
Background
Senescent-like Blood Lymphocytes and Disease Progression in Amyotrophic Lateral Sclerosis
Yildiz, O., Schroth, J., Tree, T., Turner, M. R., Shaw, P. J., Henson, S. M., & Malaspina, A. (2023) · Neurology Neuroimmunology & Neuroinflammation · doi:10.1212/nxi.0000000000200042 · Cited by 39
“Critically, other alterations observed in aging and senescence are aligned with brain-specific changes seen in ALS, including a disturbed autophagy/ lysosomal protein degradation, altered RNA splicing, and errors in nuclear-cytoplasmic transport.(15-18) We can therefore hypothesize a potential role for cell senescence…”
Background
Passive Transfer of Sera from ALS Patients with Identified Mutations Evokes an Increased Synaptic Vesicle Number and Elevation of Calcium Levels in Motor Axon Terminals, Similar to Sera from Sporadic Patients
Meszlényi, V., Patai, R., Polgár, T. F., Nógrádi, B., Körmöczy, L., Kristóf, R., Spisák, K., Tripolszki, K., Széll, M., Obál, I., et al. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21155566 · Cited by 39
“Mutations in this gene have a strong immune/inflammatory effect due to a generalized systemic pro-inflammatory state, which initiates autoimmunity-related degenerative pathways and microglia activation in the C9ORF72 knock-out model of ALS [59], without a regression of the motor function [60].”
Background
RNA Sequencing Reveals Small and Variable Contributions of Infectious Agents to Transcriptomes of Postmortem Nervous Tissues From Amyotrophic Lateral Sclerosis, Alzheimer’s Disease and Parkinson’s Disease Subjects, and Increased Expression of Genes From Disease-Activated Microglia
Bennett, J. P., Keeney, P. M., & Brohawn, D. G. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.00235 · Cited by 38
Exploring Links Between Psychosis and Frontotemporal Dementia Using Multimodal Machine Learning
Koutsouleris, N., Pantelis, C., Velakoulis, D., McGuire, P. K., Dwyer, D., Urquijo-Castro, M. F., Paul, R., Dong, S., Popovic, D., Oeztuerk, O. F., et al. (2022) · JAMA Psychiatry · doi:10.1001/jamapsychiatry.2022.2075 · Cited by 42
A Combination of Neurofilament Light, Glial Fibrillary Acidic Protein, and Neuronal Pentraxin-2 Discriminates Between Frontotemporal Dementia and Other Dementias
Bolsewig, K., Hok-A-Hin, Y. S., Sepe, F. N., Boonkamp, L., Jacobs, D., Bellomo, G., Paoletti, F. P., Vanmechelen, E., Teunissen, C. E., Parnetti, L., et al. (2022) · Journal of Alzheimer's Disease · doi:10.3233/jad-220318 · Cited by 36
Emerging Evidence Highlighting the Importance of Redox Dysregulation in the Pathogenesis of Amyotrophic Lateral Sclerosis (ALS)
Jagaraj, C. J., Parakh, S., & Atkin, J. D. (2021) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2020.581950 · Cited by 38
“Furthermore, whilst loss of C9orf72 in mice models disturbs microglial function, resulting in age-related neuroinflammation, this was not sufficient to cause neurodegeneration in C9orf72 knockout mice (Lall and Baloh, 2017).”
Background
Targeting TREM2 for Parkinson’s Disease: Where to Go?
Li, X., & Zhang, F. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2021.795036 · Cited by 38
Exploring Cerebrospinal Fluid IgG N-Glycosylation as Potential Biomarker for Amyotrophic Lateral Sclerosis
Costa, J., Streich, L., Pinto, S., Pronto‐Laborinho, A. C., Nimtz, M., Conradt, H. S., & de Carvalho, M. A. (2019) · Molecular Neurobiology · doi:10.1007/s12035-019-1482-9 · 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
“Astrocytes are found in postmortem brain tissue, in the subcortical white matter and spinal cord of both SALS and FALS patients, suggesting that astrocytes respond to CNS injury (40,41).”
BackgroundInfluential
Functional microglia neurotransmitters in amyotrophic lateral sclerosis
Volonté, C., Amadio, S., Fabbrizio, P., & Apolloni, S. (2019) · Seminars in Cell and Developmental Biology · doi:10.1016/j.semcdb.2019.04.014 · Cited by 34
Mechanisms of Immune Activation by c9orf72-Expansions in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
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
Redefining microglia states: Lessons and limits of human and mouse models to study microglia states in neurodegenerative diseases
de Soysa, Y., Therrien, M., Walker, A. C., & Stevens, B. (2022) · Seminars in Immunology · doi:10.1016/j.smim.2022.101651 · Cited by 34
Negative regulation of TREM2-mediated C9orf72 poly-GA clearance by the NLRP3 inflammasome
Shu, X., Chen, W., Tu, W.-Y., Zhong, K., Qi, S., Wang, A., Bai, L., Zhang, S.-X., Luo, B., Xu, Z., et al. (2023) · Cell Reports · doi:10.1016/j.celrep.2023.112133 · Cited by 33
ATAXIN-2 intermediate-length polyglutamine expansions elicit ALS-associated metabolic and immune phenotypes
de Sá, R. V., Sudrià-Lopez, E., Luna, M. C., Harschnitz, O., van den Heuvel, D. M. A., Kling, S., Vonk, D., Westeneng, H., Karst, H., Bloemenkamp, L. M., et al. (2024) · Nature Communications · doi:10.1038/s41467-024-51676-0 · Cited by 37
“Microglia are the resident immune cells of the CNS and are linked to various neu-rodegenerativedisorders, includingALS 57,58 .”
Stem cell transplantation for amyotrophic lateral sclerosis
Baloh, R. H., Glass, J. D., & Svendsen, C. N. (2018) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000598 · Cited by 36
Self-citation
Potential targets of microglia in the treatment of neurodegenerative diseases: Mechanism and therapeutic implications
Zhao, W., Liu, Z., Wu, J., Liu, A., & Yan, J. (2025) · Neural Regeneration Research · doi:10.4103/nrr.nrr-d-24-01343 · Cited by 37
“These cases are commonly referred to as “ALS-FTD spectrum disorders” (Lall and Baloh, 2017).”
Thrombin and the Coag-Inflammatory Nexus in Neurotrauma, ALS, and Other Neurodegenerative Disorders
Festoff, B. W., & Citron, B. A. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00059 · Cited by 31
“This situation is fundamentally the same for neuroinflammation in ALS— that is, is it cause or effect? The findings that the C9orf72 expansions are also associated with myeloid cell abnormalities and early BBB dysfunction supports the role of these processes in pathogenesis (107, 236).”
BackgroundInfluential
SMCR8 negatively regulates AKT and MTORC1 signaling to modulate lysosome biogenesis and tissue homeostasis
Lan, Y., Sullivan, P. M., & Hu, F. (2019) · Autophagy · doi:10.1080/15548627.2019.1569914 · Cited by 34
“However, autoimmunity strongly associates with ALS-FTLD [32].”
Influential
C9orf72 Intermediate Alleles in Patients with Amyotrophic Lateral Sclerosis, Systemic Lupus Erythematosus, and Rheumatoid Arthritis
Fredi, M., Cavazzana, I., Biasiotto, G., Filosto, M., Padovani, A., Monti, E., Tincani, A., Franceschini, F., & Zanella, I. (2019) · NeuroMolecular Medicine · doi:10.1007/s12017-019-08528-8 · Cited by 31
Associations between autoimmune diseases and amyotrophic lateral sclerosis: a register-based study
Cui, C., Longinetti, E., Larsson, H., Andersson, J., Yudi Pawitan, Y., Piehl, F., & Fang, F. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1861022 · Cited by 31
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
“, corticosteroids, cyclophosphamide, azathioprine, intravenous immunoglobulins, and plasmapheresis) have failed to show any efficacy in ALS patients [133] makes this area of research still challenging.”
Background
C9-ALS-Associated Proline-Arginine Dipeptide Repeat Protein Induces Activation of NLRP3 Inflammasome of HMC3 Microglia Cells by Binding of Complement Component 1 Q Subcomponent-Binding Protein (C1QBP), and Syringin Prevents This Effect
Fu, R., Tsai, C.-W., Chiu, S., Liu, S., Chiang, Y.-T., Kuo, Y.-H., Shyu, W., & Lin, S. (2022) · Cells · doi:10.3390/cells11193128 · Cited by 29
“C9-ALS has been shown to be closely related to the neuroinflammation of microglial cells [48].”
Background
Downregulation of Hsp90 and the antimicrobial peptide Mtk suppresses poly(GR)-induced neurotoxicity in C9ORF72-ALS/FTD
Lee, S., Jun, Y., Linares, G. R., Butler, B., Yuva-Adyemir, Y., Moore, J. E., Krishnan, G., Ruiz-Juarez, B., Santana, M., Pons, M., et al. (2023) · Neuron · doi:10.1016/j.neuron.2023.02.029 · Cited by 28
“Moreover, loss of C9ORF72 protein function may alter immune cell function.(43) It will be interesting to investigate how poly(GR) and the partial loss of C9ORF72 synergize to dysregulate the AMP gene pathway in neurons and glial cells.”
Amyotrophic lateral sclerosis
Younger, D. S., & Brown, R. H. (2023) · Handbook of clinical neurology · doi:10.1016/b978-0-323-98817-9.00031-4 · Cited by 35
Inflammatory neuropsychiatric disorders and COVID-19 neuroinflammation
Tang, S. W., Helmeste, D. M., & Leonard, B. E. (2021) · Acta Neuropsychiatrica · doi:10.1017/neu.2021.13 · Cited by 27
“, 2008), in which activated microglia are prominent (Lall and Baloh 2017).”
Background
Histamine beyond its effects on allergy: Potential therapeutic benefits for the treatment of Amyotrophic Lateral Sclerosis (ALS)
Volonté, C., Apolloni, S., & Sabatelli, M. (2019) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2019.06.006 · Cited by 26
Common Neurodegeneration-Associated Proteins Are Physiologically Expressed by Human B Lymphocytes and Are Interconnected via the Inflammation/Autophagy-Related Proteins TRAF6 and SQSTM1
Nataf, S., Guillen, M., & Pays, L. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.02704 · Cited by 25
Stem Cell Transplantation for Amyotrophic Lateral Sclerosis
Zhu, Q., & Lu, P. (2020) · Advances in experimental medicine and biology · doi:10.1007/978-981-15-4370-8_6 · Cited by 25
Increased Microglial CSF1R Expression in the SIV/Macaque Model of HIV CNS Disease
Knight, A. C., Brill, S. A., Queen, S. E., Tarwater, P. M., & Mankowski, J. L. (2018) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/nlx115 · Cited by 27
“Animals were either (1) mock inoculated with Lactated Ringers Solution, (2) inoculated intravenously with the neurovirulent molecular clone SIV/17E-Fr and the immunosuppressive swarm SIV/DeltaB670 (SIV), or (3) SIV inoculated and treated with an ART regimen (SIVþART) (20–2).”
Method
Identification of Anti-Neuroinflammatory Bioactive Compounds in Essential Oils and Aqueous Distillation Residues Obtained from Commercial Varieties of Cannabis sativa L.
Barbalace, M. C., Freschi, M., Rinaldi, I., Mazzara, E., Maraldi, T., Malaguti, M., Prata, C., Maggi, F., Petrelli, R., Hrelia, S., et al. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms242316601 · Cited by 24
Cerebellum in neurodegenerative diseases: Advances, challenges, and prospects
Liu, G., Yang, C., Wang, X., Chen, X., Cai, H., & Le, W. (2024) · iScience · doi:10.1016/j.isci.2024.111194 · Cited by 25
“113 Autopsy results of patients with FUS mutations show FUS expression and neurodegenerative changes in the cerebellum.”
Novel chemical inhibitor against SOD1 misfolding and aggregation protects neuron-loss and ameliorates disease symptoms in ALS mouse model
Woo, T., Yoon, M.-H., Kang, S., Park, S., Cho, J.-H., Hwang, Y. J., Ahn, J., Jang, H., Shin, Y.-J., Jung, E., et al. (2021) · Communications Biology · doi:10.1038/s42003-021-02862-z · Cited by 24
Inflammatory checkpoints in amyotrophic lateral sclerosis: From biomarkers to therapeutic targets
Jiang, Z., Wang, Z., Wei, X., & Yu, X. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.1059994 · Cited by 23
Moderate intrinsic phenotypic alterations in C9orf72 ALS/FTD iPSC-microglia despite the presence of C9orf72 pathological features
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
“As a result, neuroinflammation is considered a major contributor to neuronal dysfunction in neurodegenerative diseases, including ALS/FTD (Lall and Baloh, 2017; Beers and Appel, 2019; McCauley and Baloh, 2019; Olesen et al., 2020).”
BackgroundSelf-citation
Positive Allosteric Modulation of CB1 and CB2 Cannabinoid Receptors Enhances the Neuroprotective Activity of a Dual CB1R/CB2R Orthosteric Agonist
Polini, B., Cervetto, C., Carpi, S., Pelassa, S., Gado, F., Ferrisi, R., Bertini, S., Nieri, P., Marcoli, M., & Manera, C. (2020) · Life · doi:10.3390/life10120333 · Cited by 22
“Indeed, the microglial activation and the consequent neuroinflammation play key roles in several neurodegenerative diseases, such as Alzheimer’s disease [22], Parkinson’s disease [23], amyotrophic lateral sclerosis and frontotemporal dementia [24,25], and also, in psychiatric disorders [26].”
Background
C9ORF72 protein function and immune dysregulation in amyotrophic lateral sclerosis
Lai, J. D., & Ichida, J. K. (2019) · Neuroscience Letters · doi:10.1016/j.neulet.2019.134523 · Cited by 25
Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degenerations: Similarities in Genetic Background
Parobková, E., & Matěj, R. (2021) · Diagnostics · doi:10.3390/diagnostics11030509 · Cited by 25
Lipocalin‐2 is increased in amyotrophic lateral sclerosis
Petrozziello, T., Mills, A. N., Farhan, S. M. K., Mueller, K. A., Granucci, E. J., Glajch, K. E., Chan, J., Chew, S., Berry, J. D., & Sadri‐Vakili, G. (2020) · Muscle & Nerve · doi:10.1002/mus.26911 · Cited by 17
Dysregulation of Steroid Hormone Receptors in Motor Neurons and Glia Associates with Disease Progression in ALS Mice
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Cotinine: A Therapy for Memory Extinction in Post-traumatic Stress Disorder
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Astrocytes in Motor Neuron Diseases
Valori, C. F., Guidotti, G., Brambilla, L., & Rossi, D. (2019) · Advances in experimental medicine and biology · doi:10.1007/978-981-13-9913-8_10 · Cited by 19
Endogenous TDP-43 mislocalization in a novel knock-in mouse model reveals DNA repair impairment, inflammation, and neuronal senescence
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Prominent microglial activation in cortical white matter is selectively associated with cortical atrophy in primary progressive aphasia
Ohm, D. T., Kım, G., Gefen, T. D., RADEMAKER, A. W., Weıntraub, S., Bigio, E. H., Mesulam, M. M., Rogalski, E. J., & Geula, C. (2018) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12494 · Cited by 19
Repeat length increases disease penetrance and severity in C9orf72 ALS/FTD BAC transgenic mice
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OPTN p.Met468Arg and ATXN2 intermediate length polyQ extension in families with C9orf72 mediated amyotrophic lateral sclerosis and frontotemporal dementia
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“…autophagy, where the collapse of proteostasis machinery leads to the accumulation of misfolded proteins (Bendotti et al., 2012; Cirulli et al., 2015), or altered systemic immunity resulting in neuroinflammation (Gleason, Ordureau, Gourlay, Arthur, & Cohen, 2011; Hiscott, 2007; Lall & Baloh, 2017).”
Background
Effects of exercise on neuroinflammation in age-related neurodegenerative disorders
Su, Y., & Su, Z. (2025) · European journal of medical research · doi:10.1186/s40001-025-03165-3 · Cited by 22
“According to recent studies, ongoing and excessive stimulation of inflammasomes is thought to contribute to the development of neuroinflammation in ALS [178, 179].”

Authors: Lall, D., & Baloh, R. H.
Affiliations: Regenerative Medicine Institute (MX)
Cedars-Sinai Medical Center (US)
Corresponding author: Baloh, Robert H.
ORCID: Baloh, Robert H.
Venue: Journal of Clinical Investigation
Publisher: American Society for Clinical Investigation
Published: 2017-07-24
Volume: 127
Issue: 9
Pages: 3250-3258
ISSN: 0021-9738 (ISSN-L)
1558-8238
DOI: 10.1172/jci90607
PMC: PMC5669558
OpenAlex: W2736657964
Cited by: 175
References: 150
Citations by year: 2026: 14 · 2025: 16 · 2024: 11 · 2023: 22 · 2022: 27 · 2021: 16 · 2020: 20 · 2019: 28 · 2018: 16 · 2017: 5
FWCI: 2.50
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: Journal of Clinical Investigation (publishedVersion)
Europe PMC (PubMed Central) (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: Target ALS
Cedars-Sinai Medical Center
National Institutes of Health
National Institute of Neurological Disorders and Stroke (R01NS097545, R01NS069669)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
Health Sciences › Medicine › Clinical Neurology › Multiple Sclerosis Research Studies
Keywords: microglia, C9orf72, amyotrophic lateral sclerosis, frontotemporal dementia, neuroinflammation, autoimmunity, myeloid cells, neurodegeneration, pathogenesis, systemic immunity
MeSH terms: Superoxide Dismutase-1, C9orf72 Protein, Amyotrophic Lateral Sclerosis, Animals, DNA-Binding Proteins, Humans, Inflammation, Motor Neurons, Mutation, Phenotype, Proteins, Autoimmunity, Microglia, Disease Progression, Genetic Predisposition to Disease, Myeloid Cells, Mice, Frontotemporal Dementia

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Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia

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