Cargando…

Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity

Hexanucleotide repeat expansions in C9orf72 are the most common inherited cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The expansions elicit toxicity in part through repeat-associated non-AUG (RAN) translation of the intronic (GGGGCC)(n) sequence into dipeptide rep...

Descripción completa

Detalles Bibliográficos
Autores principales:	Flores, Brittany N., Dulchavsky, Mark E., Krans, Amy, Sawaya, Michael R., Paulson, Henry L., Todd, Peter K., Barmada, Sami J., Ivanova, Magdalena I.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2016
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5077081/ https://www.ncbi.nlm.nih.gov/pubmed/27776165 http://dx.doi.org/10.1371/journal.pone.0165084

Ejemplares similares

The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome
por: Gupta, Rahul, et al.
Publicado: (2017)

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD
por: Freibaum, Brian D., et al.
Publicado: (2017)

Toxicity of C9orf72-associated dipeptide repeat peptides is modified by commonly used protein tags
por: Morón-Oset, Javier, et al.
Publicado: (2023)

Emerging Perspectives on Dipeptide Repeat Proteins in C9ORF72 ALS/FTD
por: Schmitz, Alexander, et al.
Publicado: (2021)

RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response
por: Green, Katelyn M., et al.
Publicado: (2017)

Type I PRMT Inhibition Protects Against C9ORF72 Arginine-Rich Dipeptide Repeat Toxicity
por: Premasiri, Alan S., et al.
Publicado: (2020)

The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS/FTD
por: He, Fang, et al.
Publicado: (2020)

Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size
por: Bennion Callister, Janis, et al.
Publicado: (2016)

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics
por: Boeynaems, Steven, et al.
Publicado: (2017)

Dipeptide repeat (DPR) pathology in the skeletal muscle of ALS patients with C9ORF72 repeat expansion
por: Cykowski, Matthew D., et al.
Publicado: (2019)

Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration
por: Ohki, Yu, et al.
Publicado: (2017)

CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9orf72 dipeptide repeat protein toxicity
por: Kramer, Nicholas J., et al.
Publicado: (2018)

Antibodies inhibit transmission and aggregation of C9orf72 poly‐GA dipeptide repeat proteins
por: Zhou, Qihui, et al.
Publicado: (2017)

Differential toxicity and localization of arginine-rich C9ORF72 dipeptide repeat proteins depend on de-clustering of positive charges
por: Miyagi, Tamami, et al.
Publicado: (2023)

Reduced autophagy upon C9ORF72 loss synergizes with dipeptide repeat protein toxicity in G4C2 repeat expansion disorders
por: Boivin, Manon, et al.
Publicado: (2020)

SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72
por: Malnar, Mirjana, et al.
Publicado: (2021)

C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration
por: May, Stephanie, et al.
Publicado: (2014)

C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import
por: Hayes, Lindsey R, et al.
Publicado: (2020)

Impaired ribosome-associated quality control of C9orf72 arginine-rich dipeptide-repeat proteins
por: Viera Ortiz, Ashley P, et al.
Publicado: (2022)

CGG repeats trigger translational frameshifts that generate aggregation-prone chimeric proteins
por: Wright, Shannon E, et al.
Publicado: (2022)

Nuclear localized C9orf72-associated arginine-containing dipeptides exhibit age-dependent toxicity in C. elegans
por: Rudich, Paige, et al.
Publicado: (2017)

Unaffected mosaic C9orf72 case: RNA foci, dipeptide proteins, but upregulated C9orf72 expression
por: McGoldrick, Philip, et al.
Publicado: (2018)

Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G(4)C(2) RNA in a cellular model
por: Frottin, Frédéric, et al.
Publicado: (2021)

Reactivation of nonsense-mediated mRNA decay protects against C9orf72 dipeptide-repeat neurotoxicity
por: Xu, Wangchao, et al.
Publicado: (2019)

Interactome screening of C9orf72 dipeptide repeats reveals VCP sequestration and functional impairment by polyGA
por: Božič, Janja, et al.
Publicado: (2021)

Dipeptide Repeat Pathology in C9orf72-ALS Is Associated with Redox, Mitochondrial and NRF2 Pathway Imbalance
por: Jiménez-Villegas, José, et al.
Publicado: (2022)

The conserved multi-functional nuclear protein dss-1/Sem1 is required for C9orf72-associated ALS/FTD dipeptide toxicity
por: Puleo, Noah, et al.
Publicado: (2020)

Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing
por: Schludi, Martin H., et al.
Publicado: (2015)

CUG initiation and frameshifting enable production of dipeptide repeat proteins from ALS/FTD C9ORF72 transcripts
por: Tabet, Ricardos, et al.
Publicado: (2018)

Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
por: Mordes, Daniel A., et al.
Publicado: (2018)

Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease
por: Sakae, Nobutaka, et al.
Publicado: (2018)

C9orf72-associated arginine-rich dipeptide repeats induce RNA-dependent nuclear accumulation of Staufen in neurons
por: Kim, Eun Seon, et al.
Publicado: (2021)

Proximity proteomics of C9orf72 dipeptide repeat proteins identifies molecular chaperones as modifiers of poly-GA aggregation
por: Liu, Feilin, et al.
Publicado: (2022)

Poly-dipeptides produced from C9orf72 hexanucleotide repeats cause selective motor neuron hyperexcitability in ALS
por: Jo, Yunhee, et al.
Publicado: (2022)

Translation of dipeptide repeat proteins in C9ORF72 ALS/FTD through unique and redundant AUG initiation codons
por: Sonobe, Yoshifumi, et al.
Publicado: (2023)

Accumulation of dipeptide repeat proteins predates that of TDP‐43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene
por: Baborie, Atik, et al.
Publicado: (2015)

Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72
por: Davidson, Yvonne S, et al.
Publicado: (2014)

Drosha Inclusions Are New Components of Dipeptide-Repeat Protein Aggregates in FTLD-TDP and ALS C9orf72 Expansion Cases
por: Porta, Sílvia, et al.
Publicado: (2015)

Erratum to: Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing
por: Schludi, Martin H., et al.
Publicado: (2015)

Poly‐GP in cerebrospinal fluid links C9orf72‐associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD
por: Lehmer, Carina, et al.
Publicado: (2017)

Cannot write session to /tmp/vufind_sessions/sess_rhp9lk2bh4b8ht7mob7r6kuf6c