The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum
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HDAC6 regulates mutant SOD1 aggregation through two SMIR motifs and tubulin acetylationUnconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALSThe genetics and neuropathology of frontotemporal lobar degenerationMolecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision MedicineClinical and genetic basis of familial amyotrophic lateral sclerosisMechanisms of granulin deficiency: lessons from cellular and animal modelsCognitive and behavioral features of c9FTD/ALSPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersParkinsonian syndrome in familial frontotemporal dementiaConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisThe function of RNA-binding proteins at the synapse: implications for neurodegenerationSelf-propagation of pathogenic protein aggregates in neurodegenerative diseasesHippocampal sclerosis of aging, a prevalent and high-morbidity brain diseaseNeurodegenerative diseases: expanding the prion conceptPathogenesis of FUS-associated ALS and FTD: insights from rodent modelsTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.C9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseasesABCC9 gene polymorphism is associated with hippocampal sclerosis of aging pathology.Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth.Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLDTopology of a G-quadruplex DNA formed by C9orf72 hexanucleotide repeats associated with ALS and FTD.Trk receptor signaling and sensory neuron fate are perturbed in human neuropathy caused by Gars mutations.C9orf72 nucleotide repeat structures initiate molecular cascades of disease.Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discoveryDrosophila 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.Rare mutations in SQSTM1 modify susceptibility to frontotemporal lobar degenerationIs synaptic loss a unique hallmark of Alzheimer's disease?Reassessment of risk genotypes (GRN, TMEM106B, and ABCC9 variants) associated with hippocampal sclerosis of aging pathologyDownregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodiesG-quadruplex structures formed by expanded hexanucleotide repeat RNA and DNA from the neurodegenerative disease-linked C9orf72 gene efficiently sequester and activate heme.Rab8, POSH, and TAK1 regulate synaptic growth in a Drosophila model of frontotemporal dementiaInvestigating the role of filamin C in Belgian patients with frontotemporal dementia linked to GRN deficiency in FTLD-TDP brains.The synaptic accumulation of hyperphosphorylated tau oligomers in Alzheimer disease is associated with dysfunction of the ubiquitin-proteasome system.Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure.A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouseClinical features of TBK1 carriers compared with C9orf72, GRN and non-mutation carriers in a Belgian cohort.A pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats.Clinic, neuropathology and molecular genetics of frontotemporal dementia: a mini-review.
P2860
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P2860
The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
@zh
2012年學術文章
@zh-hant
name
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@ast
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@en
type
label
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@ast
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@en
prefLabel
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@ast
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@en
P2860
P921
P1433
P1476
The molecular basis of the fro ...... hic lateral sclerosis spectrum
@en
P2093
Tim Van Langenhove
P2860
P304
P356
10.3109/07853890.2012.665471
P407
P5008
P577
2012-03-16T00:00:00Z
2012-12-01T00:00:00Z