The RNA binding protein, TDP-43
RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy
... aetiology, TDP- 43 proteinopathy is a common pathological feature that is observed in 495% of ALS and tau-negative frontotemporal dementia (FTD) ...cases. TDP-43 is a ...
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Long non-coding RNA NEAT1_1 ameliorates TDP-43 toxicity in in vivo models of TDP-43 proteinopathy
... Introduction TDP-43 is an abundant, ubiquitously expressed RNA-binding protein [1] whose dysfunction is tightly linked to and/or causa- tive of neurodegenerative diseases amyotrophic ...
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Identification of Protein-Protein Interactions of Amyotrophic Lateral Sclerosis Associated Protein TDP-43
... DNA-binding protein (TDP-43). TDP- 43 TDP-43 is a 414-amino acid DNA and RNA binding protein that is highly conserved and ubiquitously ...
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Dysregulation of RNA Binding Protein Aggregation in Neurodegenerative Disorders
... eIF2α: Protein Kinase R (PKR), PKR-like/Pancreatic Endoplasmic Reticulum kinase (PERK), Heme-Regulated Inhibitor (HRI), or General Control Non-derepressible 2 (GCN) ( Taniuchi et ...stranded RNA, enabling ...
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TDP-35 sequesters TDP-43 into cytoplasmic inclusions through binding with RNA
... inclusion RNA recognition motif a b s t r a c t TDP-43 (TAR DNA binding protein of 43kDa) and its C-terminal fragments are thought to be linked to the pathologies of amyotrophic lateral ...
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Interdomain interactions of the transactive response DNA binding protein 43 kDa (TDP-43)
... length TDP-43 protein with greater ...the binding interface within the RNA-binding domain would be ...upon binding and activation of crosslinking, the C-terminal domain ...
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Characterizing RNA Targets of TDP-43
... f TDP-43 dependent differential protein expression using 2-Dim ensional gel ...in protein expression under different conditions is 2-Dimensional (2-DE) gel ...global protein expression ...
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Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)
... DNA-binding protein of 43 kDa (TDP-43) or Fused in sarcoma (FUS) are a hallmark of amyotrophic lateral sclerosis (ALS) and several subtypes of frontotem- poral lobar degeneration ...
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Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C
... of TDP-43 in neurodegenerative disease and fur- ther highlight the need to prioritize the targeting of this protein to develop novel therapeutic ...in RNA bind- ing proteins, especially those ...
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Tar DNA binding Protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue
... DNA Binding Protein-43 (TDP-43) is a principle component of inclusions in many cases of frontotemporal lobar degeneration (FTLD-U) and amyotrophic lateral sclerosis ...(ALS). ...
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DNA binding induces a nanomechanical switch in the RRM1 domain of TDP-43
... conditions, TDP-43 is predominantly localised in the nucleus with low levels in the cytoplasm 39-40 and conducts a multiplex of functionalities, being involved in different steps of RNA processing ...
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Analysis of ALS and FTLD-U linked protein TDP-43 in Drosophila melanogaster
... [44], TDP-43 was linked to neurodegeneration by Neumann et ...[3]. TDP-43 was identified as the major protein component of inclusions that are characteristic of most forms of ALS and ...
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Folding of the RNA recognition motif (RRM) domains of the amyotrophic lateral sclerosis (ALS)-linked protein TDP-43 reveals an intermediate state
... mented TDP-43 as well as other known protein ...of TDP-43 populates the interme- diate state, and the equilibrium heavily favors the folded TDP-43 with a properly folded ...
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Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy
... antisense RNA mole- cules might be expected to be equally toxic via their shared protein binding partners, distinct patterns of expression in various CNS neuronal populations could lead to relative ...
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High-Resolution RNA Maps Suggest Common Principles of Splicing and Polyadenylation Regulation by TDP-43
... expressRNA supports several open-source and commercial 3 0 end sequencing protocols (Lexogen forward/reverse, 3P-seq, pA-seq, PolyAseq). The platform is modular and scalable and runs on desktop computers for small ...
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High-Resolution RNA Maps Suggest Common Principles of Splicing and Polyadenylation Regulation by TDP-43
... expressRNA supports several open-source and commercial 3 0 end sequencing protocols (Lexogen forward/reverse, 3P-seq, pA-seq, PolyAseq). The platform is modular and scalable and runs on desktop computers for small ...
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Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation
... NTD-driven TDP-43 oligomerization is independent from RNA binding, although the potential increase in local TDP-43 concentration upon RNA binding within cells may ...
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RNA Processing Dysfunction in TDP-ALS
... 4 TDP-43 further contains two RRMs, both involved in controlling TDP-43 DNA and RNA binding function (Kuo et ...2009). TDP-43 was first discovered as a possible ...
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The role of ubiquitinated TDP-43 in amyotrophic lateral sclerosis
... DNA binding protein-43 (TDP-43) is the main component of intracellular ubiquitin inclusion bodies in pathological ...deposits. TDP-43 is mainly distributed in the nucleus ...
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Systemic dysregulation of TDP-43 binding microRNAs in amyotrophic lateral sclerosis
... in TDP-43 transgenic mouse models or in human ALS post-mortem tissue, wild-type TDP-43 or mutants thereof tend to re-localize from a predominant nuclear localization to the cytoplasm and ...
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