The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
about
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivoPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersPhosphorylation by PINK1 releases the UBL domain and initializes the conformational opening of the E3 ubiquitin ligase ParkinActivation of the E3 ubiquitin ligase Parkin.The crystal structure of TDP-43 RRM1-DNA complex reveals the specific recognition for UG- and TG-rich nucleic acidsThe TDP-43 N-terminal domain structure at high resolutionNimbolide targets BCL2 and induces apoptosis in preclinical models of Waldenströms macroglobulinemia.Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation.Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.TDP-43 N terminus encodes a novel ubiquitin-like fold and its unfolded form in equilibrium that can be shifted by binding to ssDNATDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodiesALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43.(Patho-)physiological relevance of PINK1-dependent ubiquitin phosphorylationRBM45 homo-oligomerization mediates association with ALS-linked proteins and stress granulesTDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD.A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defectsHeterozygous PINK1 p.G411S increases risk of Parkinson's disease via a dominant-negative mechanism.Targeting TDP-43 in neurodegenerative diseases.The Role of TDP-43 in Alzheimer's Disease.Old versus New Mechanisms in the Pathogenesis of ALS.Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions.Prion-like propagation as a pathogenic principle in frontotemporal dementia.The N-terminal dimerization is required for TDP-43 splicing activity.TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domainThe structural integrity of TDP-43 N-terminus is required for efficient aggregate entrapment and consequent loss of protein function.An Amyloid-Like Pathological Conformation of TDP-43 Is Stabilized by Hypercooperative Hydrogen Bonds.Biological Spectrum of Amyotrophic Lateral Sclerosis Prions.TDP-43 misexpression causes defects in dendritic growth.The N-Terminal Domain of ALS-Linked TDP-43 Assembles without Misfolding.TDP-43 self-interaction is modulated by redox-active compounds Auranofin, Chelerythrine and Riluzole.Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.Towards a TDP-43-Based Biomarker for ALS and FTLD.A single N-terminal phosphomimic disrupts TDP-43 polymerization, phase separation, and RNA splicing.Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies.Transactive response DNA-binding protein 43 (TDP-43) regulates alternative splicing of tau exon 10: Implications for the pathogenesis of tauopathies.Protein molecular modeling techniques investigating novel TAB2 variant R347X causing cardiomyopathy and congenital heart defects in multigenerational family.Protein molecular modeling shows residue T599 is critical to wild-type function of POLG and description of a novel variant associated with the SANDO phenotype.Whole Exome Sequencing and Molecular Modeling of a Missense Variant in TNFAIP3 That Segregates with Disease in a Family with Chronic Urticaria and Angioedema.Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo
P2860
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P2860
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
description
2013 nî lūn-bûn
@nan
2013 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2013 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
name
The dual functions of the extr ...... ivity and inclusion formation.
@ast
The dual functions of the extr ...... ivity and inclusion formation.
@en
type
label
The dual functions of the extr ...... ivity and inclusion formation.
@ast
The dual functions of the extr ...... ivity and inclusion formation.
@en
prefLabel
The dual functions of the extr ...... ivity and inclusion formation.
@ast
The dual functions of the extr ...... ivity and inclusion formation.
@en
P2093
P2860
P356
P1476
The dual functions of the extr ...... ivity and inclusion formation.
@en
P2093
Caroline Stetler
Ena C Whitelaw
Hiroki Sasaguri
Jaime Hubbard
Tania F Gendron
Thomas Caulfield
Wing Cheung Lee
Yong-Jie Zhang
P2860
P304
P356
10.1093/HMG/DDT166
P577
2013-04-10T00:00:00Z