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Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome
Over the last decades, research dedicated to the molecular and cellular mechanisms underlying primary immunodeficiencies (PID) has helped to understand the etiology of many of these diseases and to develop novel therapeutic approaches. Beyond these aspects, PID are also studied because they offer in...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4321635/ https://www.ncbi.nlm.nih.gov/pubmed/25709608 http://dx.doi.org/10.3389/fimmu.2015.00047 |
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author | Cotta-de-Almeida, Vinicius Dupré, Loïc Guipouy, Delphine Vasconcelos, Zilton |
author_facet | Cotta-de-Almeida, Vinicius Dupré, Loïc Guipouy, Delphine Vasconcelos, Zilton |
author_sort | Cotta-de-Almeida, Vinicius |
collection | PubMed |
description | Over the last decades, research dedicated to the molecular and cellular mechanisms underlying primary immunodeficiencies (PID) has helped to understand the etiology of many of these diseases and to develop novel therapeutic approaches. Beyond these aspects, PID are also studied because they offer invaluable natural genetic tools to dissect the human immune system. In this review, we highlight the research that has focused over the last 20 years on T lymphocytes from Wiskott–Aldrich syndrome (WAS) patients. WAS T lymphocytes are defective for the WAS protein (WASP), a regulator of actin cytoskeleton remodeling. Therefore, study of WAS T lymphocytes has helped to grasp that many steps of T lymphocyte activation and function depend on the crosstalk between membrane receptors and the actin cytoskeleton. These steps include motility, immunological synapse assembly, and signaling, as well as the implementation of helper, regulatory, or cytotoxic effector functions. The recent concept that WASP also works as a regulator of transcription within the nucleus is an illustration of the complexity of signal integration in T lymphocytes. Finally, this review will discuss how further study of WAS may contribute to solve novel challenges of T lymphocyte biology. |
format | Online Article Text |
id | pubmed-4321635 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-43216352015-02-23 Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome Cotta-de-Almeida, Vinicius Dupré, Loïc Guipouy, Delphine Vasconcelos, Zilton Front Immunol Immunology Over the last decades, research dedicated to the molecular and cellular mechanisms underlying primary immunodeficiencies (PID) has helped to understand the etiology of many of these diseases and to develop novel therapeutic approaches. Beyond these aspects, PID are also studied because they offer invaluable natural genetic tools to dissect the human immune system. In this review, we highlight the research that has focused over the last 20 years on T lymphocytes from Wiskott–Aldrich syndrome (WAS) patients. WAS T lymphocytes are defective for the WAS protein (WASP), a regulator of actin cytoskeleton remodeling. Therefore, study of WAS T lymphocytes has helped to grasp that many steps of T lymphocyte activation and function depend on the crosstalk between membrane receptors and the actin cytoskeleton. These steps include motility, immunological synapse assembly, and signaling, as well as the implementation of helper, regulatory, or cytotoxic effector functions. The recent concept that WASP also works as a regulator of transcription within the nucleus is an illustration of the complexity of signal integration in T lymphocytes. Finally, this review will discuss how further study of WAS may contribute to solve novel challenges of T lymphocyte biology. Frontiers Media S.A. 2015-02-09 /pmc/articles/PMC4321635/ /pubmed/25709608 http://dx.doi.org/10.3389/fimmu.2015.00047 Text en Copyright © 2015 Cotta-de-Almeida, Dupré, Guipouy and Vasconcelos. 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) or licensor 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 | Immunology Cotta-de-Almeida, Vinicius Dupré, Loïc Guipouy, Delphine Vasconcelos, Zilton Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title | Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title_full | Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title_fullStr | Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title_full_unstemmed | Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title_short | Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott–Aldrich Syndrome |
title_sort | signal integration during t lymphocyte activation and function: lessons from the wiskott–aldrich syndrome |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4321635/ https://www.ncbi.nlm.nih.gov/pubmed/25709608 http://dx.doi.org/10.3389/fimmu.2015.00047 |
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