Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
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From animal models to human disease: a genetic approach for personalized medicine in ALSHigh-Resolution RNA Maps Suggest Common Principles of Splicing and Polyadenylation Regulation by TDP-43.Aldehyde Dehydrogenases 1A2 Expression and Distribution are Potentially Associated with Neuron Death in Spinal Cord of Tg(SOD1*G93A)1Gur Mice.Drosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects.Drosophila TDP-43 RNA-Binding Protein Facilitates Association of Sister Chromatid Cohesion Proteins with Genes, Enhancers and Polycomb Response Elements.TDP-43 and FUS en route from the nucleus to the cytoplasm.Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis.Zinc binding to RNA recognition motif of TDP-43 induces the formation of amyloid-like aggregates.TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins.Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) ProteinopathiesGene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.Neuron-to-Neuron Transfer of FUS in Drosophila Primary Neuronal Culture Is Enhanced by ALS-Associated Mutations.Role of FET proteins in neurodegenerative disorders.Cajal bodies in neuronsRNA-binding proteins implicated in neurodegenerative diseases.Expression and Distribution of Arylsulfatase B are Closely Associated with Neuron Death in SOD1 G93A Transgenic Mice.Pathology of Neurodegenerative Diseases.Control of mRNA Translation in ALS ProteinopathyTDP-43 and Cytoskeletal Proteins in ALS.Autophagy and Its Impact on Neurodegenerative Diseases: New Roles for TDP-43 and C9orf72.TDP-43 in the spectrum of MND-FTLD pathologies.Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells.Profilin 1 mutants form aggregates that induce accumulation of prion-like TDP-43The minor spliceosome could be the major key for FUS/TLS mutants in ALSGenetic mutations in RNA-binding proteins and their roles in ALS.Human Endogenous Retrovirus-K and TDP-43 Expression Bridges ALS and HIV Neuropathology.TDP-43 regulates cancer-associated microRNAs.The roles of intrinsic disorder-based liquid-liquid phase transitions in the "Dr. Jekyll-Mr. Hyde" behavior of proteins involved in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on in Silico Data.mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.TIA1 variant drives myodegeneration in multisystem proteinopathy with SQSTM1 mutations.Plastic roles of phenylalanine and tyrosine residues of TLS/FUS in complex formation with the G-quadruplexes of telomeric DNA and TERRA.Frontotemporal dementia: from molecular mechanisms to therapy.RhoGAPp190: A potential player in tbph-mediated neurodegeneration in Drosophila.Loss of TDP43 inhibits progression of triple-negative breast cancer in coordination with SRSF3.Facioscapulohumeral Muscular Dystrophy.Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions.Analysis of the substrate recognition state of TDP-43 to single-stranded DNA using fluorescence correlation spectroscopy.Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
P2860
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P2860
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
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
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
@en
type
label
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
@en
prefLabel
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
@en
P2860
P356
P1476
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.
@en
P2093
Antonia Ratti
P2860
P304
P356
10.1111/JNC.13625
P407
P478
138 Suppl 1
P577
2016-03-26T00:00:00Z