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Human HEMK1 methylates all mitochondrial release factors and is required for mitochondrial function under stress conditions

GSE336475 Homo sapiens Expression profiling by high throughput sequencing 4 samples Submitted 2026/06/29 Platform GPL24676
Summary
HEMK1 is the mitochondrial homolog of HEMK2, the enzyme that methylates the conserved glutamine in the GGQ loop of eukaryotic translation termination factor eRF1. Previous work reported altered GGQ-loop glutamine methylation of mitochondrial release factors (MRF) after HEMK1 deletion, but direct biochemical evidence for HEMK1 catalytic activity and the functional consequences of MRF methylation for mitochondrial translation and physiology remained unclear. We generated HEMK1 knockout HEK293 cells and analyzed their transcriptome and proteome but detected only limited effects on the global cellular transcriptome. In contrast, we observed that many mitochondrially encoded transcripts were consistently reduced. The reduction in mitochondrial transcripts may be a secondary consequence of impaired mitochondrial translation leading to mRNA destabilization.
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Also filed as BioProject PRJNA1481688 and SRA study SRP712338. Searching any of these in the dataset finder brings you back here.

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