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.
about
Modeling ALS and FTD with iPSC-derived neuronsGlycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegenerationC9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumNeurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.Nucleocytoplasmic transport in C9orf72-mediated ALS/FTD.Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype.Old versus New Mechanisms in the Pathogenesis of ALS.Dynamic expression of the mouse orthologue of the human amyotropic lateral sclerosis associated gene C9orf72 during central nervous system development and neuronal differentiation.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies.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.Motoneuron Disease: Basic Science.RNA Misprocessing in C9orf72-Linked Neurodegeneration.Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.Biological Spectrum of Amyotrophic Lateral Sclerosis Prions.Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.Regulation of mRNA Translation in Neurons-A Matter of Life and Death.Early Cognitive, Structural, and Microstructural Changes in Presymptomatic C9orf72 Carriers Younger Than 40 Years.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.Causative Genes in Amyotrophic Lateral Sclerosis and Protein Degradation Pathways: a Link to Neurodegeneration.Cerebellar neuronal loss in amyotrophic lateral sclerosis cases with ATXN2 intermediate repeat expansions.Neurodegeneration: Problems at the nuclear pore.
P2860
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P2860
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.
description
2015 nî lūn-bûn
@nan
2015年の論文
@ja
2015年論文
@yue
2015年論文
@zh-hant
2015年論文
@zh-hk
2015年論文
@zh-mo
2015年論文
@zh-tw
2015年论文
@wuu
2015年论文
@zh
2015年论文
@zh-cn
name
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@ast
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@en
type
label
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@ast
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@en
prefLabel
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@ast
Dipeptide repeat protein inclu ...... y to cause their degeneration.
@en
P2093
P2860
P50
P1476
Dipeptide repeat protein inclu ...... ly to cause their degeneration
@en
P2093
Jorge Gomez-Deza
Matthew Nolan
Safa Al-Sarraj
P2860
P2888
P356
10.1186/S40478-015-0218-Y
P577
2015-06-25T00:00:00Z