C9orf72 is required for proper macrophage and microglial function in mice.
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There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSC9orf72's Interaction with Rab GTPases-Modulation of Membrane Traffic and AutophagyA C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagyThe ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway.Loss of C9orf72 Enhances Autophagic Activity via Deregulated mTOR and TFEB SignalingC9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling.Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune diseaseEarly microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutationImmune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy miceCytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?Pathology of callosal damage in ALS: An ex-vivo, 7 T diffusion tensor MRI studyLoss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromesA Highly Efficient Human Pluripotent Stem Cell Microglia Model Displays a Neuronal-Co-culture-Specific Expression Profile and Inflammatory Response.Poly-GP in cerebrospinal fluid links C9orf72-associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD.Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaSRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.Cell-type specific differences in promoter activity of the ALS-linked C9orf72 mouse ortholog.Network degeneration and dysfunction in presymptomatic C9ORF72 expansion carriers.Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAsIncreased prevalence of autoimmune disease within C9 and FTD/MND cohorts: Completing the picture.Forward Genetic Screen in Caenorhabditis elegans Suggests F57A10.2 and acp-4 As Suppressors of C9ORF72 Related Phenotypes.Elevated TMEM106B levels exaggerate lipofuscin accumulation and lysosomal dysfunction in aged mice with progranulin deficiencyLoss of C9orf72 function leads to autoimmunityPoly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.Hematopoietic stem and progenitor cells as novel prognostic biomarkers of longevity in a murine model for amyotrophic lateral sclerosis.Hypo- and Hyper-Assembly Diseases of RNA-Protein Complexes.Review: Induced pluripotent stem cell models of frontotemporal dementia.Precision medicine, genomics and drug discovery.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Role of the JAK/STAT signaling pathway in regulation of innate immunity in neuroinflammatory diseases.Decoding ALS: from genes to mechanism.TBK1: a new player in ALS linking autophagy and neuroinflammation.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.C9orf72: At the intersection of lysosome cell biology and neurodegenerative disease.The helminth product, ES-62 modulates dendritic cell responses by inducing the selective autophagolysosomal degradation of TLR-transducers, as exemplified by PKCδ.Protein-Remodeling Factors As Potential Therapeutics for Neurodegenerative Disease.
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P2860
C9orf72 is required for proper macrophage and microglial function in mice.
description
2016 nî lūn-bûn
@nan
2016年の論文
@ja
2016年論文
@yue
2016年論文
@zh-hant
2016年論文
@zh-hk
2016年論文
@zh-mo
2016年論文
@zh-tw
2016年论文
@wuu
2016年论文
@zh
2016年论文
@zh-cn
name
C9orf72 is required for proper macrophage and microglial function in mice.
@en
C9orf72 is required for proper macrophage and microglial function in mice.
@nl
type
label
C9orf72 is required for proper macrophage and microglial function in mice.
@en
C9orf72 is required for proper macrophage and microglial function in mice.
@nl
prefLabel
C9orf72 is required for proper macrophage and microglial function in mice.
@en
C9orf72 is required for proper macrophage and microglial function in mice.
@nl
P2093
P2860
P50
P921
P356
P1433
P1476
C9orf72 is required for proper macrophage and microglial function in mice.
@en
P2093
A K M G Muhammad
C A Dangler
H S Goodridge
J G O'Rourke
L Bogdanik
P2860
P304
P356
10.1126/SCIENCE.AAF1064
P407
P577
2016-03-01T00:00:00Z