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LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease
The pentatricopeptide repeat (PPR) family plays a major role in RNA stability, regulation, processing, splicing, translation, and editing. Leucine-rich PPR-motif-containing protein (LRPPRC), a member of the PPR family, is a known gene mutation that causes Leigh syndrome French–Canadian. Recently, gr...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543908/ https://www.ncbi.nlm.nih.gov/pubmed/31178748 http://dx.doi.org/10.3389/fphys.2019.00595 |
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author | Cui, Jie Wang, Li Ren, Xiaoyue Zhang, Yamin Zhang, Hongyi |
author_facet | Cui, Jie Wang, Li Ren, Xiaoyue Zhang, Yamin Zhang, Hongyi |
author_sort | Cui, Jie |
collection | PubMed |
description | The pentatricopeptide repeat (PPR) family plays a major role in RNA stability, regulation, processing, splicing, translation, and editing. Leucine-rich PPR-motif-containing protein (LRPPRC), a member of the PPR family, is a known gene mutation that causes Leigh syndrome French–Canadian. Recently, growing evidence has pointed out that LRPPRC dysregulation is related to various diseases ranging from tumors to viral infections. This review presents available published data on the LRPPRC protein function and its role in tumors and other diseases. As a multi-functional protein, LRPPRC regulates a myriad of biological processes, including energy metabolism and maturation and the export of nuclear mRNA. Overexpression of LRPPRC has been observed in various human tumors and is associated with poor prognosis. Downregulation of LRPPRC inhibits growth and invasion, induces apoptosis, and overcomes drug resistance in tumor cells. In addition, LRPPRC plays a potential role in Parkinson's disease, neurofibromatosis 1, viral infections, and venous thromboembolism. Further investigating these new functions of LRPPRC should provide novel opportunities for a better understanding of its pathological role in diseases from tumors to viral infections and as a potential biomarker and molecular target for disease treatment. |
format | Online Article Text |
id | pubmed-6543908 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-65439082019-06-07 LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease Cui, Jie Wang, Li Ren, Xiaoyue Zhang, Yamin Zhang, Hongyi Front Physiol Physiology The pentatricopeptide repeat (PPR) family plays a major role in RNA stability, regulation, processing, splicing, translation, and editing. Leucine-rich PPR-motif-containing protein (LRPPRC), a member of the PPR family, is a known gene mutation that causes Leigh syndrome French–Canadian. Recently, growing evidence has pointed out that LRPPRC dysregulation is related to various diseases ranging from tumors to viral infections. This review presents available published data on the LRPPRC protein function and its role in tumors and other diseases. As a multi-functional protein, LRPPRC regulates a myriad of biological processes, including energy metabolism and maturation and the export of nuclear mRNA. Overexpression of LRPPRC has been observed in various human tumors and is associated with poor prognosis. Downregulation of LRPPRC inhibits growth and invasion, induces apoptosis, and overcomes drug resistance in tumor cells. In addition, LRPPRC plays a potential role in Parkinson's disease, neurofibromatosis 1, viral infections, and venous thromboembolism. Further investigating these new functions of LRPPRC should provide novel opportunities for a better understanding of its pathological role in diseases from tumors to viral infections and as a potential biomarker and molecular target for disease treatment. Frontiers Media S.A. 2019-05-24 /pmc/articles/PMC6543908/ /pubmed/31178748 http://dx.doi.org/10.3389/fphys.2019.00595 Text en Copyright © 2019 Cui, Wang, Ren, Zhang and Zhang. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Physiology Cui, Jie Wang, Li Ren, Xiaoyue Zhang, Yamin Zhang, Hongyi LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title | LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title_full | LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title_fullStr | LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title_full_unstemmed | LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title_short | LRPPRC: A Multifunctional Protein Involved in Energy Metabolism and Human Disease |
title_sort | lrpprc: a multifunctional protein involved in energy metabolism and human disease |
topic | Physiology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543908/ https://www.ncbi.nlm.nih.gov/pubmed/31178748 http://dx.doi.org/10.3389/fphys.2019.00595 |
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