Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia

Copper-Zinc Superoxide Dismutase (SOD1) Is Released by Microglial Cells and Confers Neuroprotection against 6-OHDA Neurotoxicity

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Neurotoxic reactive astrocytes are induced by activated microglia

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

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Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

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A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

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Microglia Sculpt Postnatal Neural Circuits in an Activity and Complement-Dependent Manner

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Microglia: Active sensor and versatile effector cells in the normal and pathologic brain

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Apolipoprotein E and Alzheimer disease: risk, mechanisms and therapy

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Complement and microglia mediate early synapse loss in Alzheimer mouse models

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TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

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Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

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Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

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Decoding ALS: from genes to mechanism

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The Immunological Genome Project: networks of gene expression in immune cells

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Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

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New tools for studying microglia in the mouse and human CNS

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TREM2 Lipid Sensing Sustains the Microglial Response in an Alzheimer’s Disease Model

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Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

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TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

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Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

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Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

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ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

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Alzheimer’s Disease Risk Genes and Mechanisms of Disease Pathogenesis

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Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

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Mutations of optineurin in amyotrophic lateral sclerosis

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Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

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Microglia development follows a stepwise program to regulate brain homeostasis

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Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

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Debris clearance by microglia: an essential link between degeneration and regeneration

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Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

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Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS

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Infiltrating monocytes trigger EAE progression, but do not contribute to the resident microglia pool

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The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.

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C9orf72 nucleotide repeat structures initiate molecular cascades of disease

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Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

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The changing scene of amyotrophic lateral sclerosis

Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957 · see citing works

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

Rosen, D. (1993) · Nature · doi:10.1038/364362c0 · Cited by 922 · see citing works

Origin and differentiation of microglia

Ginhoux, F., Lim, S., Hoeffel, G., Low, D., & Huber, T. (2013) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2013.00045 · Cited by 905 · cited 1× · see citing works

RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention

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GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

Freibaum, B. D., Lu, Y., Lopez-Gonzalez, R., Kim, N. C., Almeida, S., Lee, K.-H., Badders, N., Valentine, M., Miller, B. L., Wong, P. C., et al. (2015) · Nature · doi:10.1038/nature14974 · Cited by 841 · see citing works

Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

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TREM2 mutations implicated in neurodegeneration impair cell surface transport and phagocytosis

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Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

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Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study

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Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

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Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation

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TREMs in the immune system and beyond

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Natural history of amyotrophic lateral sclerosis in a database population Validation of a scoring system and a model for survival prediction

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Targeting RNA Foci in iPSC-Derived Motor Neurons from ALS Patients with a <i>C9ORF72</i> Repeat Expansion

Sareen, D., O’Rourke, J. G., Meera, P., Muhammad, A. K. M. G., Grant, S., Simpkinson, M., Bell, S., Carmona, S., Ornelas, L., Sahabian, A., et al. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3007529 · Cited by 670 · see citing works

&lt;emph type="ital"&gt;SQSTM1&lt;/emph&gt; Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

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Microglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis

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A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study

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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

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Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

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Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

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Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration

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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

Gendron, T. F., Bieniek, K. F., Zhang, Y., Jansen‐West, K. R., Ash, P. E. A., Caulfield, T. R., Daughrity, L. M., Dunmore, J., Castanedes‐Casey, M., Chew, J., et al. (2013) · Acta Neuropathologica · doi:10.1007/s00401-013-1192-8 · Cited by 569 · see citing works

New insights on the role of microglia in synaptic pruning in health and disease

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Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

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Presence of dendritic cells, MCP‐1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord tissue

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<i>C9orf72</i> is required for proper macrophage and microglial function in mice

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Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic

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Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment

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Repeat expansion disease: progress and puzzles in disease pathogenesis

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C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

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ApoE and Clusterin Cooperatively Suppress Aβ Levels and Deposition

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The microglial "activation" continuum: from innate to adaptive responses

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Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS

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Immune reactivity in a mouse model of familial ALS correlates with disease progression

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Accumulation of SOD1 Mutants in Postnatal Motoneurons Does Not Cause Motoneuron Pathology or Motoneuron Disease

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Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death

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Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

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SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model

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Microglia in ALS: The Good, The Bad, and The Resting

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Microglia Function in Central Nervous System Development and Plasticity

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Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

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Implications of Microglia in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

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