Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis
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A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusionsRapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instabilityMolecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision MedicineTherapeutic and diagnostic challenges for frontotemporal dementiaDoes a loss of TDP-43 function cause neurodegeneration?TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.A 43-kDa TDP-43 species is present in aggregates associated with frontotemporal lobar degenerationTARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathyTDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degenerationLoss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis.Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy.ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis.Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasmWild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.Astrocytic TDP-43 pathology in Alexander diseaseNovel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumOverexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanismUbiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS).Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chainsTransactive response DNA-binding protein 43 burden in familial Alzheimer disease and Down syndromeThe role of transactive response DNA-binding protein-43 in amyotrophic lateral sclerosis and frontotemporal dementia.TAR DNA-binding protein 43 in neurodegenerative disease.Neurotoxic effects of TDP-43 overexpression in C. elegans.Frontotemporal lobar degeneration: epidemiology, pathophysiology, diagnosis and management.ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injuryAmyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.Asparagine endopeptidase is an innovative therapeutic target for neurodegenerative diseases.Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.TDP-43 in aging and Alzheimer's disease - a reviewReview: transactive response DNA-binding protein 43 (TDP-43): mechanisms of neurodegeneration.Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathiesA "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transport.Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies.Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine.
P2860
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P2860
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@ast
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@en
type
label
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@ast
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@en
prefLabel
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@ast
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@en
P2093
P2860
P50
P1476
Enrichment of C-terminal fragm ...... amyotrophic lateral sclerosis
@en
P2093
Adam C Truax
Christopher M Clark
Kunihiro Uryu
Lauren B Elman
Leo F McCluskey
Linda K Kwong
Lionel M Igaz
Virginia M-Y Lee
P2860
P304
P356
10.2353/AJPATH.2008.080003
P407
P577
2008-06-05T00:00:00Z