(Q42693496)

4 November 2017

title

Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS. (English)

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amyotrophic lateral sclerosis

4 November 2017

main subject

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2

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4 November 2017

6

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4 November 2017

7

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4 November 2017

Ken-ichiro Kubo

4

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author name string

Mai Yamakawa

series ordinal

1

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4 November 2017

Takao Honda

series ordinal

3

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4 November 2017

Mariko Noda

series ordinal

5

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4 November 2017

language of work or name

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14 November 2014

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4 November 2017

published in

Human Molecular Genetics

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4 November 2017

volume

24

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4 November 2017

issue

6

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4 November 2017

page(s)

1630-1645

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Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS

4 November 2017

cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Modelling C9ORF72 hexanucleotide repeat expansion in amyotrophic lateral sclerosis and frontotemporal dementia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Ubiquitinated p62-positive, TDP-43-negative inclusions in cerebellum in frontotemporal lobar degeneration with TAR DNA binding protein 43.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Tau pathology in frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansion

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Arginine-rich motifs present multiple interfaces for specific binding by RNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9ORF72 hexanucleotide repeat expansions in the Italian sporadic ALS population

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Labeling embryonic mouse central nervous system cells by in utero electroporation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Efficient in utero gene transfer system to the developing mouse brain using electroporation: visualization of neuronal migration in the developing cortex.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Beyond laminar fate: toward a molecular classification of cortical projection/pyramidal neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Transcriptional regulation of cortical neuron migration by POU domain factors

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Brn-1 and Brn-2 share crucial roles in the production and positioning of mouse neocortical neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A minimal glycine-alanine repeat prevents the interaction of ubiquitinated I kappaB alpha with the proteasome: a new mechanism for selective inhibition of proteolysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Inhibition of proteasomal degradation by the gly-Ala repeat of Epstein-Barr virus is influenced by the length of the repeat and the strength of the degradation signal

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Enhancement of proteasome activity by a small-molecule inhibitor of USP14

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A simple method for displaying the hydropathic character of a protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Stress granules as crucibles of ALS pathogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Diffusible, nonfibrillar ligands derived from Abeta1-42 are potent central nervous system neurotoxins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A specific amyloid-beta protein assembly in the brain impairs memory

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Endoplasmic reticulum stress is important for the manifestations of α-synucleinopathy in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Alpha-synuclein: from secretion to dysfunction and death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Inhibition of ubiquitin-dependent proteolysis by a synthetic glycine-alanine repeat peptide that mimics an inhibitory viral sequence

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Inhibition of ubiquitin/proteasome-dependent proteolysis in Saccharomyces cerevisiae by a Gly-Ala repeat

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

C9ORF72 expansion is not a significant cause of sporadic spinocerebellar ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

The role of autophagy during the early neonatal starvation period

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Fractalkine attenuates excito-neurotoxicity via microglial clearance of damaged neurons and antioxidant enzyme heme oxygenase-1 expression

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

A schizophrenia-associated mutation of DISC1 perturbs cerebral cortex development

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU576

21 January 2018

Identifiers

DOI

10.1093/HMG/DDU576

1 reference

4 November 2017

1 reference