Cargando…

The RNA helicase DHX36–G4R1 modulates C9orf72 GGGGCC hexanucleotide repeat–associated translation

GGGGCC (G(4)C(2)) hexanucleotide repeat expansions in the endosomal trafficking gene C9orf72 are the most common genetic cause of ALS and frontotemporal dementia. Repeat-associated non-AUG (RAN) translation of this expansion through near-cognate initiation codon usage and internal ribosomal entry ge...

Descripción completa

Detalles Bibliográficos
Autores principales:	Tseng, Yi-Ju, Sandwith, Siara N., Green, Katelyn M., Chambers, Antonio E., Krans, Amy, Raimer, Heather M., Sharlow, Meredith E., Reisinger, Michael A., Richardson, Adam E., Routh, Eric D., Smaldino, Melissa A., Wang, Yuh-Hwa, Vaughn, James P., Todd, Peter K., Smaldino, Philip J.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Society for Biochemistry and Molecular Biology 2021
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8326427/ https://www.ncbi.nlm.nih.gov/pubmed/34174288 http://dx.doi.org/10.1016/j.jbc.2021.100914

Ejemplares similares

G-Quadruplex Helicase DHX36/G4R1 Engages Nuclear Lamina Proteins in Quiescent Breast Cancer Cells
por: Richardson, Adam. E., et al.
Publicado: (2020)

DNA Replication Dynamics of the GGGGCC Repeat of the C9orf72 Gene
por: Thys, Ryan Griffin, et al.
Publicado: (2015)

C9ORF72 Gene GGGGCC Hexanucleotide Expansion: A High Clinical Variability from Amyotrophic Lateral Sclerosis to Frontotemporal Dementia
por: Kortazar-Zubizarreta, Izaro, et al.
Publicado: (2023)

Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia
por: Fratta, Pietro, et al.
Publicado: (2013)

Processing of double-R-loops in (CAG)·(CTG) and C9orf72 (GGGGCC)·(GGCCCC) repeats causes instability
por: Reddy, Kaalak, et al.
Publicado: (2014)

Ribosome profiling reveals novel regulation of C9ORF72 GGGGCC repeat-containing RNA translation
por: van ‘t Spijker, Heleen M., et al.
Publicado: (2022)

Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72
por: Boeve, Bradley F., et al.
Publicado: (2012)

Genetic counseling for FTD/ALS caused by the C9ORF72 hexanucleotide expansion
por: Fong, Jamie C, et al.
Publicado: (2012)

Molecular Mechanisms of Neurodegeneration Related to C9orf72 Hexanucleotide Repeat Expansion
por: Babić Leko, Mirjana, et al.
Publicado: (2019)

Expanding the Spectrum of Movement Disorders Associated With C9orf72 Hexanucleotide Expansions
por: Estevez-Fraga, Carlos, et al.
Publicado: (2021)

C9orf72 hexanucleotide repeat allele tagging SNPs: Associations with ALS risk and longevity
por: Kaivola, Karri, et al.
Publicado: (2023)

Pathological assessments for the presence of hexanucleotide repeat expansions in C9ORF72 in Alzheimer’s disease
por: Davidson, Yvonne S, et al.
Publicado: (2013)

Improved PCR based methods for detecting C9orf72 hexanucleotide repeat expansions
por: Cleary, Elaine M., et al.
Publicado: (2016)

Proposed association between the hexanucleotide repeat of C9orf72 and opposability index of the thumb
por: Chen, Zhongbo, et al.
Publicado: (2017)

No Association of Multiple Sclerosis with C9orf72 Hexanucleotide Repeat Size in an Austrian Cohort
por: König, Theresa, et al.
Publicado: (2023)

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

Analysis of the GGGGCC Repeat Expansions of the C9orf72 Gene in SCA3/MJD Patients from China
por: Wang, Chunrong, et al.
Publicado: (2015)

Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.
por: Goodman, Lindsey D., et al.
Publicado: (2019)

Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features
por: Mahoney, Colin J., et al.
Publicado: (2012)

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

Knock in of a hexanucleotide repeat expansion in the C9orf72 gene induces ALS in rats
por: Dong, Wei, et al.
Publicado: (2020)

C9orf72 Hexanucleotide Repeat in Huntington-Like Patients: Systematic Review and Meta-Analysis
por: Alva-Diaz, Carlos, et al.
Publicado: (2020)

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

C9orf72 Hexanucleotide Repeat Expansion-Related Neuropathology Is Attenuated by Nasal Rifampicin in Mice
por: Hatanaka, Yukari, et al.
Publicado: (2022)

G-Quadruplexes Formation by the C9orf72 Nucleotide Repeat Expansion d(GGGGCC)(n) and Conformation Regulation by Fangchinoline
por: Zhang, Yun, et al.
Publicado: (2023)

Retention of hexanucleotide repeat-containing intron in C9orf72 mRNA: implications for the pathogenesis of ALS/FTD
por: Niblock, Michael, et al.
Publicado: (2016)

High frequency of the expanded C9ORF72 hexanucleotide repeat in familial and sporadic Greek ALS patients
por: Mok, Kin Y., et al.
Publicado: (2012)

Hexanucleotide Repeat Expansion in C9ORF72 Is Not Detected in the Treatment-Resistant Schizophrenia Patients of Chinese Han
por: Xu, Xijia, et al.
Publicado: (2015)

Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal Dementia
por: Batra, Ranjan, et al.
Publicado: (2017)

Biomarkers for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Associated With Hexanucleotide Expansion Mutations in C9orf72
por: Floeter, Mary Kay, et al.
Publicado: (2018)

Somatic expansion of the C9orf72 hexanucleotide repeat does not occur in ALS spinal cord tissues
por: Ross, Jay P., et al.
Publicado: (2019)

The RNA exosome complex degrades expanded hexanucleotide repeat RNA in C9orf72 FTLD/ALS
por: Kawabe, Yuya, et al.
Publicado: (2020)

Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion()
por: Waite, Adrian J., et al.
Publicado: (2014)

C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis
por: Cooper-Knock, Johnathan, et al.
Publicado: (2015)

C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation
por: Cheng, Weiwei, et al.
Publicado: (2018)

A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD
por: Hukema, Renate K, et al.
Publicado: (2014)

Topology of a G-quadruplex DNA formed by C9orf72 hexanucleotide repeats associated with ALS and FTD
por: Zhou, Bo, et al.
Publicado: (2015)

C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes
por: Fratta, Pietro, et al.
Publicado: (2012)

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

ALS in Finland: Major Genetic Variants and Clinical Characteristics of Patients With and Without the C9orf72 Hexanucleotide Repeat Expansion
por: Laaksovirta, Hannu, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_amb978jd2qpcd6fpuv7k4bj2ic