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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...

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Autores principales: Cui, Jie, Wang, Li, Ren, Xiaoyue, Zhang, Yamin, Zhang, Hongyi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
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.
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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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