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The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3
Proteins related to the ubiquitin-proteasome system play an important role during the differentiation and ciliogenesis of photoreceptor cells. Mutations in several genes involved in ubiquitination and proteostasis have been identified as causative of inherited retinal dystrophies (IRDs) and ciliopat...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9603860/ https://www.ncbi.nlm.nih.gov/pubmed/36293380 http://dx.doi.org/10.3390/ijms232012527 |
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author | Sánchez-Bellver, Laura Férriz-Gordillo, Andrea Carrillo-Pz, Marc Rabanal, Laura Garcia-Gonzalo, Francesc R. Marfany, Gemma |
author_facet | Sánchez-Bellver, Laura Férriz-Gordillo, Andrea Carrillo-Pz, Marc Rabanal, Laura Garcia-Gonzalo, Francesc R. Marfany, Gemma |
author_sort | Sánchez-Bellver, Laura |
collection | PubMed |
description | Proteins related to the ubiquitin-proteasome system play an important role during the differentiation and ciliogenesis of photoreceptor cells. Mutations in several genes involved in ubiquitination and proteostasis have been identified as causative of inherited retinal dystrophies (IRDs) and ciliopathies. USP48 is a deubiquitinating enzyme whose role in the retina is still unexplored although previous studies indicate its relevance for neurosensory organs. In this work, we describe that a pool of endogenous USP48 localises to the basal body in retinal cells and provide data that supports the function of USP48 in the photoreceptor cilium. We also demonstrate that USP48 interacts with the IRD-associated proteins ARL3 and UNC119a, and stabilise their protein levels using different mechanisms. Our results suggest that USP48 may act in the regulation/stabilisation of key ciliary proteins for photoreceptor function, in the modulation of intracellular protein transport, and in ciliary trafficking to the photoreceptor outer segment. |
format | Online Article Text |
id | pubmed-9603860 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96038602022-10-27 The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 Sánchez-Bellver, Laura Férriz-Gordillo, Andrea Carrillo-Pz, Marc Rabanal, Laura Garcia-Gonzalo, Francesc R. Marfany, Gemma Int J Mol Sci Article Proteins related to the ubiquitin-proteasome system play an important role during the differentiation and ciliogenesis of photoreceptor cells. Mutations in several genes involved in ubiquitination and proteostasis have been identified as causative of inherited retinal dystrophies (IRDs) and ciliopathies. USP48 is a deubiquitinating enzyme whose role in the retina is still unexplored although previous studies indicate its relevance for neurosensory organs. In this work, we describe that a pool of endogenous USP48 localises to the basal body in retinal cells and provide data that supports the function of USP48 in the photoreceptor cilium. We also demonstrate that USP48 interacts with the IRD-associated proteins ARL3 and UNC119a, and stabilise their protein levels using different mechanisms. Our results suggest that USP48 may act in the regulation/stabilisation of key ciliary proteins for photoreceptor function, in the modulation of intracellular protein transport, and in ciliary trafficking to the photoreceptor outer segment. MDPI 2022-10-19 /pmc/articles/PMC9603860/ /pubmed/36293380 http://dx.doi.org/10.3390/ijms232012527 Text en © 2022 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 | Article Sánchez-Bellver, Laura Férriz-Gordillo, Andrea Carrillo-Pz, Marc Rabanal, Laura Garcia-Gonzalo, Francesc R. Marfany, Gemma The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title | The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title_full | The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title_fullStr | The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title_full_unstemmed | The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title_short | The Deubiquitinating Enzyme USP48 Interacts with the Retinal Degeneration-Associated Proteins UNC119a and ARL3 |
title_sort | deubiquitinating enzyme usp48 interacts with the retinal degeneration-associated proteins unc119a and arl3 |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9603860/ https://www.ncbi.nlm.nih.gov/pubmed/36293380 http://dx.doi.org/10.3390/ijms232012527 |
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