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

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

ORGANISM(S): Homo sapiens

PROVIDER: GSE121394 | GEO | 2022/12/22

REPOSITORIES: GEO

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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.

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

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

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

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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.

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Ribosomal protein S5b is essential for oogenesis and is required to maintain mitochondrial integrity and function in Drosophila melanogaster

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