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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 | GSE178759 | GEO

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

2021-06-29 | GSE178760 | 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

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

Gene expression response to DUX4 in murine myoblasts

Project description:DUX4 is generally agreed to underlie pathology in Facioscapulohumeral muscular dystrophy Microarrays were used to examine gene expression after primary murine satellite cell-derived myoblasts were retrovirally infected with constructs encoding DUX4, DUX4c, constitutively active, dominant negative and truncated DUX4 versions.

2017-01-31 | GSE77100 | GEO

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

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

DUX4-r exerts a neomorphic activity that depens on GTF2I in acute lymphoblastic leukemia

Project description:Translocations producing rearranged versions of the transcription factor DUX4 (DUX4-r) are one of the most frequent causes of B-cell acute lymphoblastic leukemia (B-ALL). DUX4-r retains the DNA binding domain of wt DUX4, but is truncated on the C-terminal transcription activation domain. The precise mechanism through which DUX4-r causes leukemia is unknown and no targeted therapy is currently available. We found that the rearrangement leads to both a loss and a gain of function in DUX4-r. Loss of CBP/EP300 transcriptional co-activators interaction and inability to bind and activate repressed chromatin. Gain of interaction with the transcription factor GTF2I, which redirects DUX4-r toward leukemogenic targets. Importantly, this neomorphic activity exposes an Achilles' heel whereby DUX4-r positive leukemia cells are exquisitely sensitive to GTF2I targeting, which inhibits DUX4-r leukemogenic activity. Our work elucidates the molecular mechanism through which DUX4-r causes leukemia and suggest a possible therapeutic avenue tailored to this B-ALL subtype.

2023-09-16 | PXD041273 | Pride

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