TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
about
Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolinesRecent progress in the discovery of small molecules for the treatment of amyotrophic lateral sclerosis (ALS)Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instabilityMitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Age-correlated gene expression in normal and neurodegenerative human brain tissuesBiomarkers for cognitive impairment and dementia in elderly people.Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusionsTransgenic rat model of neurodegeneration caused by mutation in the TDP gene.Elucidating the genetics and pathology of Perry syndrome.Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.Development of a novel nonradiometric assay for nucleic acid binding to TDP-43 suitable for high-throughput screening using AlphaScreen technology.Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.Profiling the genes affected by pathogenic TDP-43 in astrocytesMolecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLSTAR DNA-binding protein 43 in neurodegenerative disease.Variations in the progranulin gene affect global gene expression in frontotemporal lobar degenerationElevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.Neurodegenerative models in Drosophila: polyglutamine disorders, Parkinson disease, and amyotrophic lateral sclerosis.Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitinationOligoclonal bands in the cerebrospinal fluid of amyotrophic lateral sclerosis patients with disease-associated mutations.Nuclear localization sequence of FUS and induction of stress granules by ALS mutants.Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum.TDP-43 in aging and Alzheimer's disease - a reviewThe complexities of p97 function in health and diseaseAmyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies.Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.TDP-43 variants of frontotemporal lobar degeneration.Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation.Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin.Frontal cortex neuropathology in dementia pugilistica.Cdc37/Hsp90 protein complex disruption triggers an autophagic clearance cascade for TDP-43 proteinCharacterization of a series of 4-aminoquinolines that stimulate caspase-7 mediated cleavage of TDP-43 and inhibit its function.Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43Design, Synthesis, and Properties of Phosphoramidate 2',5'-Linked Branched RNA: Toward the Rational Design of Inhibitors of the RNA Lariat Debranching EnzymeNew directions for frontotemporal dementia drug discovery.Stress granules as crucibles of ALS pathogenesis.SQSTM1 mutations in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies.
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P2860
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
description
2007 nî lūn-bûn
@nan
2007 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@ast
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@en
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@nl
type
label
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@ast
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@en
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@nl
prefLabel
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@ast
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@en
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@nl
P2093
P2860
P3181
P1476
TDP-43 proteinopathy: the neur ...... ation and motor neuron disease
@en
P2093
Deepak M Sampathu
Linda K Kwong
Virginia M-Y Lee
P2860
P2888
P3181
P356
10.1007/S00401-007-0226-5
P407
P577
2007-07-01T00:00:00Z
P6179
1014297644