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MetabolomicsRibosomal mistranslation leads to silencing of the unfolded protein response and increased mitochondrial biogenesis

Translation fidelity is the limiting factor in the accuracy of gene expression. With an estimated frequency of 10-4, errors in mRNA decoding occur in a mostly stochastic manner. Little is known about the response of higher eukaryotes to chronic loss of ribosomal accuracy as per an increase in the...

2019-09-17 | MTBLS106 | MetaboLights
TranscriptomicsRibosomal Protein Gene Deletions in Diamond Blackfan Anemia
Diamond Blackfan anemia is a congenital bone marrow failure syndrome characterized by hypoproliferative anemia, often with associated physical abnormalities. Perturbations of the ribosome appear critically important to the development of DBA, as alterations in 9 different ribosomal protein genes ha...
ORGANISM(S): Homo sapiens 
2012-01-15 | E-GEOD-31575 | biostudies-arrayexpress
TranscriptomicsGenome-wide search for novel human uORFs and N-terminal protein extensions using ribosomal footprinting
So far, the annotation of translation initiation sites (TISs) has been based mostly upon bioinformatics rather than experimental evidence. We adapted ribosomal footprinting to puromycin-treated cells to generate a transcriptome-wide map of TISs in a human monocytic cell line. A neural network was tr...
ORGANISM(S): Homo sapiens 
2012-08-08 | E-GEOD-39561 | biostudies-arrayexpress
TranscriptomicsRibosome stalling induced by mutation of a CNS-specific tRNA causes neurodegeneration
In higher eukaryotes, the large numbers of nuclear-encoded tRNA genes partially ensure the robustness of cytoplasmic protein translation. Here we discover that a loss-of-function in n-Tr20, a member of the nuclear-encoded tRNA Arg UCU family that is expressed specifically in the central nervous syst...
ORGANISM(S): Mus musculus 
2014-07-28 | E-GEOD-56127 | biostudies-arrayexpress
ProteomicsRibosomal protein L5 (RPL5/uL18) I60V mutation is associated to increased translation and modulates drug sensitivity in T-cell acute lymphoblastic leukemia cells
Somatic mutations in ribosomal proteins (RPs), including RPL5, have been reported in approximately 10% of pediatric patients with T-cell acute lymphoblastic leukemia (T-ALL). In cancer, the incorporation of mutant RPs into ribosomes often disrupts canonical ribosome function, thereby contributing to...
ORGANISM(S): Homo sapiens (Human) 
2025-11-06 | PXD064727 | Pride
Ribosomes Target therapy Cancer Lc-ms/ms 
ProteomicsLow dose ribosomal DNA P-loop mutation affects development and enforces autophagy in Arabidopsis
Arabidopsis contains hundreds of ribosomal DNA copies organised within the nucleolar organising regions (NORs) in chromosomes 2 and 4. There are four major types of variants of rDNA, VAR1-4, based on the polymorphisms of 3' external transcribed sequences and these are variably expressed during plant...
ORGANISM(S): Arabidopsis thaliana (Mouse-ear cress) 
2024-01-26 | PXD035623 | Pride
Autophagy / crispr / development / plants / rrna 
GenomicsTargeted resequencing of ribosomal proteins in multiple myeloma patient samples.
We studied 38 multiple myeloma samples to discover mutations in ribosomal proteins. To this end, we ran a specifically designed targeted resequencing study to resequence the coding regions of all ribosomal proteins.
EGAS00001002405 | EGA
Transcriptomicsrpl24b/WT polysome loading and transcript levels (Arabidopsis thaliana)
To understand the contribution of the RPL24B protein, a component of the large 60S ribosomal subunit, to the translation of specific mRNAs, we compared the ribosome occupancy of mRNAs in wild type Arabidopsis and the rpl24b/stv1-1 T-DNA insertion mutant. RNA was fractionated using sucrose gradients ...
ORGANISM(S): Arabidopsis thaliana 
2014-01-01 | E-GEOD-51474 | biostudies-arrayexpress
UnknownThe ribosomal basis of Diamond-Blackfan Anemia: mutation and database update.
Not available
S-EPMC4485435 | biostudies-literature
GenomicsLow dose ribosomal DNA P-loop mutation affects development and enforces autophagy in Arabidopsis
Low dose ribosomal DNA P-loop mutation affects development and enforces autophagy in Arabidopsis
PRJNA882394 | ENA
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