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Chunk #0 — Introduction

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DALRD3 encodes a protein mutated in epileptic encephalopathy that targets arginine tRNAs for 3-methylcytosine modification.
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The proper maturation and function of tRNAs has emerged as a critical modulator of biological processes ranging from gene regulation to development1–3. In particular, tRNAs are subject to a diverse range of chemical modifications that play major roles in their folding, stability and function4–6. The critical role of tRNA modification in organismal physiology and fitness is highlighted by the numerous human diseases that have been associated with defects in tRNA modification including neurological disorders, mitochondrial pathologies, and cancer (reviewed in refs. 7–12).