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Human Mitochondrial RNA Processing and Modifications: Overview

期刊

出版社

MDPI
DOI: 10.3390/ijms22157999

关键词

mitochondria; mitochondrial transcription; RNA processing; RNA decay; RNA modifications; mitochondrial genome

资金

  1. Foundation for Polish Science [POIR.04.04.00-00-5E63/18-00]
  2. European Union under the European Regional Development Fund

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Mitochondria, known as the powerhouses of cells, play crucial roles in energy production, apoptosis, calcium homeostasis, and regulation of the immune response. Abnormalities in mitochondria can lead to severe diseases, making it important to study their functions and physiological consequences. Despite progress in understanding RNA processing in mitochondria, there are still many unknowns and controversies in the field.
Mitochondria, often referred to as the powerhouses of cells, are vital organelles that are present in almost all eukaryotic organisms, including humans. They are the key energy suppliers as the site of adenosine triphosphate production, and are involved in apoptosis, calcium homeostasis, and regulation of the innate immune response. Abnormalities occurring in mitochondria, such as mitochondrial DNA (mtDNA) mutations and disturbances at any stage of mitochondrial RNA (mtRNA) processing and translation, usually lead to severe mitochondrial diseases. A fundamental line of investigation is to understand the processes that occur in these organelles and their physiological consequences. Despite substantial progress that has been made in the field of mtRNA processing and its regulation, many unknowns and controversies remain. The present review discusses the current state of knowledge of RNA processing in human mitochondria and sheds some light on the unresolved issues.

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