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Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse brain tissue.

ABSTRACT: Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse brain tissue.

PROVIDER: PRJNA497257 | ENA |

REPOSITORIES: ENA

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Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse brain tissue.

Project description:Analysis of brain from C57BL/6 mice expressing V338Y mutant mitochondrial ribosomal protein.

2021-03-24 | GSE121395 | GEO

Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse muscle tissue

Project description:Analysis of muscle from C57BL/6 mice expressing V338Y mutant mitochondrial ribosomal protein.

2021-01-29 | GSE107520 | GEO

Transcriptome analysis of V336Y mutant mitochondrial ribosomal protein in human HEK293 cell line

Project description:Analysis of HEK293 cells lines expressing V336Y mutant mitochondrial ribosomal protein.

2018-10-26 | GSE101503 | GEO

Transcriptome analysis of V336Y mutant and G313R mutant mitochondrial ribosomal protein in human HEK293 cell line

Project description:Analysis of HEK293 cells lines expressing V336Y mutant and G313R mutant mitochondrial ribosomal protein

2022-12-22 | GSE121394 | GEO

Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse muscle tissue

Project description:Transcriptome analysis of V338Y mutant mitochondrial ribosomal protein in mouse muscle tissue

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Transcriptome analysis of V336Y mutant mitochondrial ribosomal protein in human HEK293 cell line

Project description:Transcriptome analysis of V336Y mutant mitochondrial ribosomal protein in human HEK293 cell line

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KDM5-mediated transcriptional activation of ribosomal protein genes alters translation efficiency to regulate mitochondrial metabolism.

Project description:Genes encoding the KDM5 family of transcriptional regulators are disrupted in individuals with intellectual disability (ID). To understand the link between KDM5 and ID, we generated five Drosophila strains harboring missense alleles analogous to those observed in patients. These alleles showed effects on neuroanatomical development, cognition, and other behaviors, in addition to a transcriptional signature that included the downregulation of many ribosomal protein genes. A similar transcriptional profile was observed in KDM5C knockout human glutamatergic neurons derived from induced pluripotent stem cells (iPSCs), suggesting a conserved role for KDM5 proteins in regulating ribosomal protein genes. Loss of ribosomal protein gene expression resulted in changes to neuronal ribosome composition. Moreover, we find that the translation efficiency of mRNAs required for mitochondrial metabolism was particularly affected upon reduction KDM5 in Drosophila neurons. Altered mitochondrial activity was confirmed through metabolomic studies that revealed decreased citric acid cycle activity. KDM5 therefore plays a key role in maintaining mitochondrial function that, when altered, could contribute to cognitive and behavioral phenotypes.

2023-11-08 | GSE245380 | GEO

KDM5-mediated transcriptional activation of ribosomal protein genes alters translation efficiency to regulate mitochondrial metabolism II.

2023-10-23 | GSE245515 | GEO

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

Project description: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 random error rate of mRNA decoding. Here, we present a global and comprehensive picture of the cellular changes in response to translational accuracy in mammalian ribosomes impaired by genetic manipulation. In addition to affecting established protein quality control pathways, such as elevated transcript levels for cytosolic chaperones, activation of the ubiquitin-proteasome system, and translational slowdown, ribosomal mistranslation led to unexpected responses. In particular, we observed increased mitochondrial biogenesis associated with import of misfolded proteins into the mitochondria and silencing of the unfolded protein response in the endoplasmic reticulum. This study describes the metabolomic analysis of HEK293 cells lines expressing mutant ribosomal protein RPS2 (human A226Y). RPS2 A226Y mutation has been shown to cause misreading and readthrough. Results provide insight into the response to chronic mistranslation in mammalian cells.

2019-09-17 | MTBLS106 | MetaboLights

BmATAD3A mediates mitochondrial ribosomal protein expression to maintain the mitochondrial energy metabolism of the silkworm Bombyx mori

Project description:ATAD3A is a mitochondrial membrane protein in the ATPase family containing an AAA+ domain, which is widely involved in mitochondrial metabolism, protein transport, cell growth, development, and other important life processes. It has previously been reported that the deletion of ATAD3A causes individual growth and development defects in humans, mice, and Caenorhabditis elegans. To further analyze the mechanism of ATAD3A defects affecting individual development, we constructed a BmATAD3A defect model in silkworm larvae to provide a reference for ATAD3A genetic diseases and molecular regulation mechanisms. The results showed that knockout of the BmATAD3A gene could significantly affect individual weight, survival rate, ATPase production, and mitochondrial metabolism after 24 h of individual incubation. Metabonomics and transcriptomics combined analysis further showed that knockout of BmATAD3A could inhibit individual amino acid biosynthesis by regulating mitochondrial ribosomal protein expression. Meanwhile, the knockout of BmATAD3A could block mitochondrial activity and ATPase synthesis through the mitochondrial ribosomal protein BmmRPL11 and thereby inhibit mitochondrial oxidative phosphorylation. These results could provide new insights into the inhibition of individual development caused by ATAD3A deficiency, thus allowing the identification of new directions for the targeted therapy of diseases caused by abnormal expression of ATAD3A.

2023-02-27 | PXD040409 | iProX

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