Bi-allelic variants in the mitochondrial RNase P subunit PRORP cause mitochondrial tRNA processing defects and pleiotropic multisystem presentations
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DOI: 10.1016/j.ajhg.2021.10.002
Abstract: Summary Human mitochondrial RNase P (mt-RNase P) is responsible for 5′ end processing of mitochondrial precursor tRNAs, a vital step in mitochondrial RNA maturation, and is comprised of three protein subunits: TRMT10C, SDR5C1 (HSD10), and… read more here.
Keywords: processing; rnase; trna processing; mitochondrial rnase ... See more keywords
Cicada Endosymbionts Have tRNAs That Are Correctly Processed Despite Having Genomes That Do Not Encode All of the tRNA Processing Machinery
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DOI: 10.1128/mbio.01950-18
Abstract: The smallest bacterial genomes, in the range of about 0.1 to 0.5 million base pairs, are commonly found in the nutritional endosymbionts of insects. These tiny genomes are missing genes that encode proteins and RNAs… read more here.
Keywords: trna; trna processing; trnas correctly; correctly processed ... See more keywords
Aberrant tRNA processing causes an autoinflammatory syndrome responsive to TNF inhibitors
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DOI: 10.1136/annrheumdis-2017-212401
Abstract: Objectives To characterise the clinical features, immune manifestations and molecular mechanisms in a recently described autoinflammatory disease caused by mutations in TRNT1, a tRNA processing enzyme, and to explore the use of cytokine inhibitors in… read more here.
Keywords: processing causes; processing; tnf; trna processing ... See more keywords
tRNA Processing and Subcellular Trafficking Proteins Multitask in Pathways for Other RNAs
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DOI: 10.3389/fgene.2019.00096
Abstract: This article focuses upon gene products that are involved in tRNA biology, with particular emphasis upon post-transcriptional RNA processing and nuclear-cytoplasmic subcellular trafficking. Rather than analyzing these proteins solely from a tRNA perspective, we explore… read more here.
Keywords: trna biology; processing subcellular; rna; subcellular trafficking ... See more keywords
A tRNA processing enzyme is a key regulator of the mitochondrial unfolded protein response.
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DOI: 10.7554/elife.71634
Abstract: The mitochondrial unfolded protein response (UPRmt) has emerged as a predominant mechanism that preserves mitochondrial function. Consequently, multiple pathways likely exist to modulate UPRmt. We discovered that the tRNA processing enzyme, homolog of ELAC2 (HOE-1),… read more here.
Keywords: response; unfolded protein; trna processing; protein response ... See more keywords