A functional connection between dyskerin and energy metabolism
| Autor: | Nunzia Matrone, Alberto Angrisani, Valentina Belli, Nunzia Di Maio, Antonio Porcellini, Filippo Scialò, Mimmo Turano, Paolo A. Netti, Rosario Vicidomini, Maria Furia |
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| Přispěvatelé: | Angrisani, Alberto, Matrone, Nunzia, Belli, Valentina, Vicidomini, Rosario, Di Maio, Nunzia, Turano, Mimmo, Scialò, Filippo, Netti, Paolo Antonio, Porcellini, Antonio, Furia, Maria, Angrisani, A., Matrone, N., Belli, V., Vicidomini, R., Di Maio, N., Turano, M., Scialo, F., Netti, P. A., Porcellini, A., Furia, M. |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Gene isoform Short Communication Clinical Biochemistry Ribosome biogenesis Cell Cycle Proteins Biology HeLa Cell Biochemistry Dyskerin 03 medical and health sciences Superoxides Cell Cycle Protein Humans Protein Isoforms Nuclear protein Small nucleolar RNA lcsh:QH301-705.5 Nuclear Protein Genetics lcsh:R5-920 Organic Chemistry Protein Isoform Superoxide Nuclear Proteins Energy metabolism DKC1 Cell biology Telomere Mitochondria ROS signaling 030104 developmental biology lcsh:Biology (General) RNA splicing PRDX-2 lcsh:Medicine (General) Reactive Oxygen Specie Reactive Oxygen Species Nuclear localization sequence Human HeLa Cells Signal Transduction |
| Zdroj: | Redox Biology Redox Biology, Vol 14, Iss, Pp 557-565 (2018) |
| Popis: | The human DKC1 gene encodes dyskerin, an evolutionarily conserved nuclear protein whose overexpression represents a common trait of many types of aggressive sporadic cancers. As a crucial component of the nuclear H/ACA snoRNP complexes, dyskerin is involved in a variety of essential processes, including telomere maintenance, splicing efficiency, ribosome biogenesis, snoRNAs stabilization and stress response. Although multiple minor dyskerin splicing isoforms have been identified, their functions remain to be defined. Considering that low-abundance splice variants could contribute to the wide functional repertoire attributed to dyskerin, possibly having more specialized tasks or playing significant roles in changing cell status, we investigated in more detail the biological roles of a truncated dyskerin isoform that lacks the C-terminal nuclear localization signal and shows a prevalent cytoplasmic localization. Here we show that this dyskerin variant can boost energy metabolism and improve respiration, ultimately conferring a ROS adaptive response and a growth advantage to cells. These results reveal an unexpected involvement of DKC1 in energy metabolism, highlighting a previously underscored role in the regulation of metabolic cell homeostasis. Graphical abstract fx1 Highlights • Human dyskerin is an evolutionary conserved component of nuclear H/ACA snoRNPs. • The functional role of a truncated dyskerin isoform (Iso3) is analyzed. • Iso3 overexpression boosts energy metabolism and induces a ROS adaptive response. • Iso3 connects dyskerin with mitochondrial functionality and redox homeostasis. |
| Databáze: | OpenAIRE |
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