Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
about
There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSModeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Drosophila as an In Vivo Model for Human Neurodegenerative DiseaseIntrinsic disorder in proteins involved in amyotrophic lateral sclerosis.Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degenerationCytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration.FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusionsCerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers.Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencingDistinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.C9orf72 mutations do not influence the tau signature of amyotrophic lateral sclerosis with cognitive impairment (ALSci).Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.Poly-dipeptides encoded by the C9ORF72 repeats block global protein translationC9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins.The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the ProteasomeThe small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.RAN translation-What makes it run?Genetics of Frontotemporal Lobar Degeneration: From the Bench to the Clinic.Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Decoding ALS: from genes to mechanism.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.CGG Repeat-Associated Non-AUG Translation Utilizes a Cap-Dependent Scanning Mechanism of Initiation to Produce Toxic Proteins.Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutationModifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS.Regulation of mRNA Translation in Neurons-A Matter of Life and Death.Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome.The Genetics of Monogenic Frontotemporal Dementia.A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.Non-AUG translation: a new start for protein synthesis in eukaryotes.C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.In Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment.C-terminal short arginine/serine repeat sequence-dependent regulation of Y14 (RBM8A) localization.Cerebellar neuronal loss in amyotrophic lateral sclerosis cases with ATXN2 intermediate repeat expansions.
P2860
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P2860
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
description
2014 nî lūn-bûn
@nan
2014年の論文
@ja
2014年論文
@yue
2014年論文
@zh-hant
2014年論文
@zh-hk
2014年論文
@zh-mo
2014年論文
@zh-tw
2014年论文
@wuu
2014年论文
@zh
2014年论文
@zh-cn
name
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@en
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@nl
type
label
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@en
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@nl
prefLabel
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@en
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@nl
P2093
P2860
P50
P356
P1476
Characterization of the dipept ...... lar pathogenesis of c9FTD/ALS.
@en
P2093
Mai Yamakawa
Mariko Noda
Takao Honda
P2860
P304
P356
10.1093/HMG/DDU576
P577
2014-11-14T00:00:00Z