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

By S. Boillée, Koji Yamanaka, C. Lobsiger, Neal G. Copeland, Nancy A. Jenkins, G. Kassiotis, G. Kollias, D. Cleveland. Science, 2006

1,646 citations recorded by the source corpus.

View Paper: https://www.semanticscholar.org/paper/625ba6e087ec52b9e6db21594f54574d6926e158

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1. M. Pekny
2. M. Pekna
3. Yuanyuan Tang
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5. R. Ransohoff
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Citations in the corpus

• Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. (2010, unknown): “onset, but do influence disease progression [77,78].”
• Astrocyte reactivity and reactive astrogliosis: costs and benefits. (2014, background): “More recent data suggest that at least in some forms of ALS, the neuronal damage is mediated by oligodendrocytes (111), microglia (25, 253), and astrocytes (57, 88, 155, 268), which thus emerge as a novel therapeutic target for this devastating disease.”
• A neurodegeneration-specific gene expression signature and immune profile of acutely isolated microglia from an ALS mouse model (2013, unknown): “CD11bCre and LysM-Cre transgenic mice have been used to study microglia (Boillée et al., 2006; Cho et al., 2008), but these genes are also expressed by peripheral myeloid immune cells.”
• Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis (2013, unknown): “…et al., 2009; Yamanaka et al., 2008) and NG2+ oligodendrocyte precursors (Kang et al., 2013), while mutant SOD1 synthesized within two additional glial cell types (astrocytes; Yamanaka et al., 2008; and microglia; Boillé e et al., 2006) are primary determinants of accelerated disease progression.”
• Microglia induce motor neuron death via the classical NF-κB pathway in amyotrophic lateral sclerosis (2014, unknown): “Landmark studies have shown selective removal of the causal mutant SOD1 gene from myeloid cells in the slow-progressing SOD1-G37R mice (Boillé e et al., 2006) or repopulating the fastprogressing SOD1-G93A model with WT myeloid cells delays disease progression (Beers et al., 2006).”
• Innate immunity in the central nervous system. (2012, unknown): “In a genetic mouse model of motor neuron disease, targeted deletion of the causative mutant superoxide dismutase gene in microglia remarkably prolonged the lifespan of the mice even though the mutant transgene was still expressed by neurons and astrocytes (62).”