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UPF1—From mRNA Degradation to Human Disorders

Up-frameshift protein 1 (UPF1) plays the role of a vital controller for transcripts, ready to react in the event of an incorrect translation mechanism. It is well known as one of the key elements involved in mRNA decay pathways and participates in transcript and protein quality control in several di...

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Autores principales: Staszewski, Jacek, Lazarewicz, Natalia, Konczak, Julia, Migdal, Iwona, Maciaszczyk-Dziubinska, Ewa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9914065/
https://www.ncbi.nlm.nih.gov/pubmed/36766761
http://dx.doi.org/10.3390/cells12030419
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author Staszewski, Jacek
Lazarewicz, Natalia
Konczak, Julia
Migdal, Iwona
Maciaszczyk-Dziubinska, Ewa
author_facet Staszewski, Jacek
Lazarewicz, Natalia
Konczak, Julia
Migdal, Iwona
Maciaszczyk-Dziubinska, Ewa
author_sort Staszewski, Jacek
collection PubMed
description Up-frameshift protein 1 (UPF1) plays the role of a vital controller for transcripts, ready to react in the event of an incorrect translation mechanism. It is well known as one of the key elements involved in mRNA decay pathways and participates in transcript and protein quality control in several different aspects. Firstly, UPF1 specifically degrades premature termination codon (PTC)-containing products in a nonsense-mediated mRNA decay (NMD)-coupled manner. Additionally, UPF1 can potentially act as an E3 ligase and degrade target proteins independently from mRNA decay pathways. Thus, UPF1 protects cells against the accumulation of misfolded polypeptides. However, this multitasking protein may still hide many of its functions and abilities. In this article, we summarize important discoveries in the context of UPF1, its involvement in various cellular pathways, as well as its structural importance and mutational changes related to the emergence of various pathologies and disease states. Even though the state of knowledge about this protein has significantly increased over the years, there are still many intriguing aspects that remain unresolved.
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spelling pubmed-99140652023-02-11 UPF1—From mRNA Degradation to Human Disorders Staszewski, Jacek Lazarewicz, Natalia Konczak, Julia Migdal, Iwona Maciaszczyk-Dziubinska, Ewa Cells Review Up-frameshift protein 1 (UPF1) plays the role of a vital controller for transcripts, ready to react in the event of an incorrect translation mechanism. It is well known as one of the key elements involved in mRNA decay pathways and participates in transcript and protein quality control in several different aspects. Firstly, UPF1 specifically degrades premature termination codon (PTC)-containing products in a nonsense-mediated mRNA decay (NMD)-coupled manner. Additionally, UPF1 can potentially act as an E3 ligase and degrade target proteins independently from mRNA decay pathways. Thus, UPF1 protects cells against the accumulation of misfolded polypeptides. However, this multitasking protein may still hide many of its functions and abilities. In this article, we summarize important discoveries in the context of UPF1, its involvement in various cellular pathways, as well as its structural importance and mutational changes related to the emergence of various pathologies and disease states. Even though the state of knowledge about this protein has significantly increased over the years, there are still many intriguing aspects that remain unresolved. MDPI 2023-01-27 /pmc/articles/PMC9914065/ /pubmed/36766761 http://dx.doi.org/10.3390/cells12030419 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Staszewski, Jacek
Lazarewicz, Natalia
Konczak, Julia
Migdal, Iwona
Maciaszczyk-Dziubinska, Ewa
UPF1—From mRNA Degradation to Human Disorders
title UPF1—From mRNA Degradation to Human Disorders
title_full UPF1—From mRNA Degradation to Human Disorders
title_fullStr UPF1—From mRNA Degradation to Human Disorders
title_full_unstemmed UPF1—From mRNA Degradation to Human Disorders
title_short UPF1—From mRNA Degradation to Human Disorders
title_sort upf1—from mrna degradation to human disorders
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9914065/
https://www.ncbi.nlm.nih.gov/pubmed/36766761
http://dx.doi.org/10.3390/cells12030419
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