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Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

ABSTRACT: Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

PROVIDER: PRJNA740300 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR14890809.fastq.gz	Fastqsanger.gz
	SRR14890810.fastq.gz	Fastqsanger.gz
	SRR14890811.fastq.gz	Fastqsanger.gz
	SRR14890812.fastq.gz	Fastqsanger.gz
	SRR14890813.fastq.gz	Fastqsanger.gz

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Similar Datasets

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

Project description:Nonsense-mediated RNA decay (NMD) degrades transcripts carrying premature termination codons. NMD is thought to prevent the synthesis of toxic truncated proteins. However, whether loss of NMD results in widespread production of truncated proteins is unclear. A human genetic disease, facioscapulohumeral muscular dystrophy (FSHD), features acute inhibition of NMD upon expression of the disease-causing transcription factor, DUX4. Using a cell-based model of FSHD, we show production of truncated proteins from physiological NMD targets and find that RNA-binding proteins are enriched for aberrant truncations. The NMD isoform of one RNA-binding protein, SRSF3, is translated to produce a stable truncated protein, which is detected in FSHD patient-derived myotubes. Ectopic expression of truncated SRSF3 confers toxicity, and its downregulation is cytoprotective. Our results delineate the genome-scale impact of NMD loss. This widespread production of potentially deleterious truncated proteins has implications for FSHD biology as well as other genetic diseases where NMD is therapeutically modulated.

2021-06-29 | GSE178760 | GEO

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

2021-06-29 | GSE178759 | GEO

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

Project description:Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

| PRJNA740298 | ENA

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

Project description:Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

| PRJNA740301 | ENA

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression.

| S-EPMC10592454 | biostudies-literature

Nonsense-mediated decay in Plasmodium falciparum

Project description:We have HA-tagged two nonsense-mediated decay proteins, UPF1 and UPF2, in the malaria parasite Plasmodium falciparum. We then performed co-immunoprecipitation experiments to determine the protein-protein interactions of these nonsense-mediated decay components.

2021-04-16 | PXD023910 | Pride

Compromised nonsense-mediated RNA decay results in truncated RNA-binding protein production upon DUX4 expression

Project description:This SuperSeries is composed of the SubSeries listed below.

2021-06-29 | GSE178761 | GEO

RNAi knock down of known nonsense-mediated mRNA decay (NMD) factors in Drosophila cells

Project description:All eukaryotes studied to date have the capacity to detect and degrade mRNAs harboring premature translation termination codons (PTCs) in a process called nonsense-mediated mRNA decay (NMD) (reviewed in Wagner E & Lykke-Andersen J, 2002). This surveillance system allows the cell to prevent the expression of potentially harmful truncated proteins. trans-acting factors required for NMD in Drosophila are the proteins UPF1, UPF2, UPF3, SMG-1, SMG-5 and SMG-6. To identify mRNAs naturally regulated by NMD, we analyzed expression profiles in Drosophila cells RNAi-depleted of known NMD factors using high-density oligonucleotide arrays.

2005-11-01 | E-MEXP-202 | biostudies-arrayexpress

Ultraconserved elements are associated with control of splicing regulators by alt splicing and nonsense mediated decay

Project description:We blocked nonsense-mediated mRNA decay (NMD) pathway and used a custom alternative transcript sensitive Affymetrix microarray to seek alternatively spliced RNAs whose levels increased compared to controls. Keywords: Nonsense Mediated Decay (NMD)

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Regulated endocleavage balances the degradation of nonsense-mediated decay substrates

Project description:Nonsense-mediated mRNA decay (NMD) controls gene expression by eliminating mRNAs with premature or aberrant translation termination. Degradation of NMD substrates is initiated by the central NMD factor UPF1, which recruits the endonuclease SMG6 and the deadenylation-promoting SMG5/7 complex. Here we map transcriptome-wide sites of SMG6-mediated endocleavage via 3′ fragment capture and degradome sequencing.

2017-06-01 | E-MTAB-3625 | biostudies-arrayexpress

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