Cargando…

Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides

An intronic GGGGCC repeat expansion in C9orf72 is a common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. The repeats are transcribed in both sense and antisense directions to generate distinct dipeptide repeat proteins, of which poly(GA), poly(GR), and poly(PR) have bee...

Descripción completa

Detalles Bibliográficos
Autores principales:	Balendra, Rubika, Ruiz de los Mozos, Igor, Odeh, Hana M, Glaria, Idoia, Milioto, Carmelo, Wilson, Katherine M, Ule, Agnieszka M, Hallegger, Martina, Masino, Laura, Martin, Stephen, Patani, Rickie, Shorter, James, Ule, Jernej, Isaacs, Adrian M
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Life Science Alliance LLC 2023
Materias:	Research Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10338859/ https://www.ncbi.nlm.nih.gov/pubmed/37438085 http://dx.doi.org/10.26508/lsa.202201824

Ejemplares similares

Soluble and insoluble dipeptide repeat protein measurements in C9orf72-frontotemporal dementia brains show regional differential solubility and correlation of poly-GR with clinical severity
por: Quaegebeur, Annelies, et al.
Publicado: (2020)

Specific biomarkers for C9orf72 FTD/ALS could expedite the journey towards effective therapies
por: Balendra, Rubika, et al.
Publicado: (2017)

Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration
por: Mizielinska, Sarah, et al.
Publicado: (2017)

C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A
por: Moens, Thomas G., et al.
Publicado: (2019)

TDP-43 condensation properties specify its RNA-binding and regulatory repertoire
por: Hallegger, Martina, et al.
Publicado: (2021)

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

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

G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo
por: Simone, Roberto, et al.
Publicado: (2017)

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

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

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

CDK11 is required for transcription of replication-dependent histone genes
por: Gajdušková, Pavla, et al.
Publicado: (2020)

Enhanced insulin signalling ameliorates C9orf72 hexanucleotide repeat expansion toxicity in Drosophila
por: Atilano, Magda L, et al.
Publicado: (2021)

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

Major Shifts in Glial Regional Identity Are a Transcriptional Hallmark of Human Brain Aging
por: Soreq, Lilach, et al.
Publicado: (2017)

Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity
por: Flores, Brittany N., et al.
Publicado: (2016)

Characterization of membrane penetration and cytotoxicity of C9orf72-encoding arginine-rich dipeptides
por: Kanekura, Kohsuke, et al.
Publicado: (2018)

C9orf72-derived arginine-rich poly-dipeptides impede phase modifiers
por: Nanaura, Hitoki, et al.
Publicado: (2021)

C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation
por: Licata, Nausicaa V, et al.
Publicado: (2021)

Alu elements: at the crossroads between disease and evolution
por: Ule, Jernej
Publicado: (2013)

Open access, open data and peer review
por: Ule, Jernej
Publicado: (2020)

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

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

C9orf72 poly(PR) mediated neurodegeneration is associated with nucleolar stress
por: Cicardi, M.E., et al.
Publicado: (2023)

Charting DENR-dependent translation reinitiation uncovers predictive uORF features and links to circadian timekeeping via Clock
por: Castelo-Szekely, Violeta, et al.
Publicado: (2019)

Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis
por: Tyzack, Giulia E, et al.
Publicado: (2019)

Splicing repression allows the gradual emergence of new Alu-exons in primate evolution
por: Attig, Jan, et al.
Publicado: (2016)

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

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

C9orf72-Derived Proline:Arginine Poly-Dipeptides Modulate Cytoskeleton and Mechanical Stress Response
por: Shiota, Tomo, et al.
Publicado: (2022)

C9orf72 dipeptides disrupt the nucleocytoplasmic transport machinery and cause TDP-43 mislocalisation to the cytoplasm
por: Ryan, Sarah, et al.
Publicado: (2022)

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

Residual association at C9orf72 suggests an alternative amyotrophic lateral sclerosis-causing hexanucleotide repeat
por: Jones, Ashley R., et al.
Publicado: (2013)

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

Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ALS/FTD is rescued by SV2 replenishment
por: Jensen, Brigid K, et al.
Publicado: (2020)

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

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

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)

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)

Cannot write session to /tmp/vufind_sessions/sess_cimphkq0uiu5u2nhqeqdqvm5p9