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Signal Recognition Particle in Human Diseases

The signal recognition particle (SRP) is a ribonucleoprotein complex with dual functions. It co-translationally targets proteins with a signal sequence to the endoplasmic reticulum (ER) and protects their mRNA from degradation. If SRP is depleted or cannot recognize the signal sequence, then the Reg...

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Autores principales: Kellogg, Morgana K., Tikhonova, Elena B., Karamyshev, Andrey L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9214365/
https://www.ncbi.nlm.nih.gov/pubmed/35754847
http://dx.doi.org/10.3389/fgene.2022.898083
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author Kellogg, Morgana K.
Tikhonova, Elena B.
Karamyshev, Andrey L.
author_facet Kellogg, Morgana K.
Tikhonova, Elena B.
Karamyshev, Andrey L.
author_sort Kellogg, Morgana K.
collection PubMed
description The signal recognition particle (SRP) is a ribonucleoprotein complex with dual functions. It co-translationally targets proteins with a signal sequence to the endoplasmic reticulum (ER) and protects their mRNA from degradation. If SRP is depleted or cannot recognize the signal sequence, then the Regulation of Aberrant Protein Production (RAPP) is activated, which results in the loss of secretory protein mRNA. If SRP recognizes the substrates but is unable to target them to ER, they may mislocalize or degrade. All these events lead to dramatic consequence for protein biogenesis, activating protein quality control pathways, and creating pressure on cell physiology, and might lead to the pathogenesis of disease. Indeed, SRP dysfunction is involved in many different human diseases, including: congenital neutropenia; idiopathic inflammatory myopathy; viral, protozoal, and prion infections; and cancer. In this work, we analyze diseases caused by SRP failure and discuss their possible molecular mechanisms.
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spelling pubmed-92143652022-06-23 Signal Recognition Particle in Human Diseases Kellogg, Morgana K. Tikhonova, Elena B. Karamyshev, Andrey L. Front Genet Genetics The signal recognition particle (SRP) is a ribonucleoprotein complex with dual functions. It co-translationally targets proteins with a signal sequence to the endoplasmic reticulum (ER) and protects their mRNA from degradation. If SRP is depleted or cannot recognize the signal sequence, then the Regulation of Aberrant Protein Production (RAPP) is activated, which results in the loss of secretory protein mRNA. If SRP recognizes the substrates but is unable to target them to ER, they may mislocalize or degrade. All these events lead to dramatic consequence for protein biogenesis, activating protein quality control pathways, and creating pressure on cell physiology, and might lead to the pathogenesis of disease. Indeed, SRP dysfunction is involved in many different human diseases, including: congenital neutropenia; idiopathic inflammatory myopathy; viral, protozoal, and prion infections; and cancer. In this work, we analyze diseases caused by SRP failure and discuss their possible molecular mechanisms. Frontiers Media S.A. 2022-06-08 /pmc/articles/PMC9214365/ /pubmed/35754847 http://dx.doi.org/10.3389/fgene.2022.898083 Text en Copyright © 2022 Kellogg, Tikhonova and Karamyshev. https://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 Genetics
Kellogg, Morgana K.
Tikhonova, Elena B.
Karamyshev, Andrey L.
Signal Recognition Particle in Human Diseases
title Signal Recognition Particle in Human Diseases
title_full Signal Recognition Particle in Human Diseases
title_fullStr Signal Recognition Particle in Human Diseases
title_full_unstemmed Signal Recognition Particle in Human Diseases
title_short Signal Recognition Particle in Human Diseases
title_sort signal recognition particle in human diseases
topic Genetics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9214365/
https://www.ncbi.nlm.nih.gov/pubmed/35754847
http://dx.doi.org/10.3389/fgene.2022.898083
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