Glycine-alanine dipeptide repeats spread rapidly in a repeat length- and age-dependent manner in the fly brain

scientific article published on 16 December 2019

Glycine-alanine dipeptide repeats spread rapidly in a repeat length- and age-dependent manner in the fly brain is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1186/S40478-019-0860-X
P932PMC publication ID6916080
P698PubMed publication ID31843021

P50authorLinda PartridgeQ6551889
Sebastian GrönkeQ40637956
Adrian M IsaacsQ88683796
Jacqueline EsserQ114722797
P2093author name stringJavier Morón-Oset
Tessa Supèr
P2860cites workFrequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional studyQ24600803
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALSQ24608159
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALSQ24633692
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTDQ24634583
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisQ26863439
Depletion of microglia and inhibition of exosome synthesis halt tau propagationQ27311815
Spreading of pathology in neurodegenerative diseases: a focus on human studiesQ28082279
An optimized transgenesis system for Drosophila using germ-line-specific phiC31 integrasesQ29615147
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.Q29871482
Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.Q33614771
Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansionsQ33784052
A conditional tissue-specific transgene expression system using inducible GAL4.Q33947918
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.Q34326849
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transportQ34491033
Optimization of dietary restriction protocols in DrosophilaQ35103827
Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cellsQ35695216
Trinucleotide repeats and neurodegenerative disease.Q35871287
Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.Q36108576
Transcellular spreading of huntingtin aggregates in the Drosophila brainQ36120432
TDP-43 is intercellularly transmitted across axon terminalsQ36310560
Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure.Q36414413
The Glycine-Alanine Dipeptide Repeat from C9orf72 Hexanucleotide Expansions Forms Toxic Amyloids Possessing Cell-to-Cell Transmission PropertiesQ36650259
Accumulation of dipeptide repeat proteins predates that of TDP-43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 geneQ37068280
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteinsQ37094833
Poly-dipeptides encoded by the C9ORF72 repeats block global protein translationQ37178589
Stages of pTDP-43 pathology in amyotrophic lateral sclerosisQ37202139
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALSQ37316045
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci.Q37316053
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementiaQ37409251
Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort studyQ37429235
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteinsQ37476206
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxicQ37503127
Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD).Q37678074
Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementiaQ38377569
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicityQ38921570
The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.Q39396328
The C9orf72 repeat size correlates with onset age of disease, DNA methylation and transcriptional downregulation of the promoterQ40415783
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriersQ40535548
Seed-induced acceleration of amyloid-β mediated neurotoxicity in vivoQ41316757
Chimeric proteins tagged with specific 3xHA cassettes may present instability and functional problemsQ41363496
Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTD.Q41570439
Antibodies inhibit transmission and aggregation of C9orf72 poly-GA dipeptide repeat proteinsQ41943289
Alzheimer brain-derived tau oligomers propagate pathology from endogenous tauQ41979763
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS.Q42925023
Spatiotemporal rescue of memory dysfunction in DrosophilaQ44681267
Mapping Wolbachia distributions in the adult Drosophila brainQ45883356
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteinsQ46797980
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.Q46799396
The molecular basis of odor coding in the Drosophila larvaQ47070576
Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disabilityQ47548592
In Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment.Q49344570
Ageing as a risk factor for ALS/FTD.Q49608524
Unconventional secretion of misfolded proteins promotes adaptation to proteasome dysfunction in mammalian cells.Q51294087
Living synaptic vesicle marker: synaptotagmin-GFP.Q52599439
Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivoQ57476533
Relations between C9orf72 expansion size in blood, age at onset, age at collection and transmission across generations in patients and presymptomatic carriersQ58553781
A feedback loop between dipeptide-repeat protein, TDP-43 and karyopherin-α mediates C9orf72-related neurodegenerationQ58698146
C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1AQ63859498
Heterochromatin anomalies and double-stranded RNA accumulation underlie poly(PR) toxicityQ64264556
C9ORF72 dipeptide repeat poly-GA inclusions promote intracellular aggregation of phosphorylated TDP-43Q88639239
Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosisQ89243459
Experimental evidence for the age dependence of tau protein spread in the brainQ93091651
P4510describes a project that usesImageJQ1659584
P433issue1
P304page(s)209
P577publication date2019-12-16
P1433published inActa Neuropathologica CommunicationsQ27724912
P1476titleGlycine-alanine dipeptide repeats spread rapidly in a repeat length- and age-dependent manner in the fly brain
P478volume7

Search more.