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The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity

Adaptor molecules lack enzymatic and transcriptional activities. Instead, they exert their function by linking multiple proteins into intricate complexes, allowing for transmitting and fine-tuning of signals. Many adaptor molecules play a crucial role in T-cell signaling, following engagement of the...

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Autores principales: Dinur-Schejter, Yael, Zaidman, Irina, Mor-Shaked, Hagar, Stepensky, Polina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8397411/
https://www.ncbi.nlm.nih.gov/pubmed/34456914
http://dx.doi.org/10.3389/fimmu.2021.701704
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author Dinur-Schejter, Yael
Zaidman, Irina
Mor-Shaked, Hagar
Stepensky, Polina
author_facet Dinur-Schejter, Yael
Zaidman, Irina
Mor-Shaked, Hagar
Stepensky, Polina
author_sort Dinur-Schejter, Yael
collection PubMed
description Adaptor molecules lack enzymatic and transcriptional activities. Instead, they exert their function by linking multiple proteins into intricate complexes, allowing for transmitting and fine-tuning of signals. Many adaptor molecules play a crucial role in T-cell signaling, following engagement of the T-cell receptor (TCR). In this review, we focus on Linker of Activation of T cells (LAT) and SH2 domain-containing leukocyte protein of 76 KDa (SLP-76). Monogenic defects in these adaptor proteins, with known roles in T-cell signaling, have been described as the cause of human inborn errors of immunity (IEI). We describe the current knowledge based on defects in cell lines, murine models and human patients. Germline mutations in Adhesion and degranulation adaptor protein (ADAP), have not resulted in a T-cell defect.
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spelling pubmed-83974112021-08-28 The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity Dinur-Schejter, Yael Zaidman, Irina Mor-Shaked, Hagar Stepensky, Polina Front Immunol Immunology Adaptor molecules lack enzymatic and transcriptional activities. Instead, they exert their function by linking multiple proteins into intricate complexes, allowing for transmitting and fine-tuning of signals. Many adaptor molecules play a crucial role in T-cell signaling, following engagement of the T-cell receptor (TCR). In this review, we focus on Linker of Activation of T cells (LAT) and SH2 domain-containing leukocyte protein of 76 KDa (SLP-76). Monogenic defects in these adaptor proteins, with known roles in T-cell signaling, have been described as the cause of human inborn errors of immunity (IEI). We describe the current knowledge based on defects in cell lines, murine models and human patients. Germline mutations in Adhesion and degranulation adaptor protein (ADAP), have not resulted in a T-cell defect. Frontiers Media S.A. 2021-08-12 /pmc/articles/PMC8397411/ /pubmed/34456914 http://dx.doi.org/10.3389/fimmu.2021.701704 Text en Copyright © 2021 Dinur-Schejter, Zaidman, Mor-Shaked and Stepensky 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 Immunology
Dinur-Schejter, Yael
Zaidman, Irina
Mor-Shaked, Hagar
Stepensky, Polina
The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title_full The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title_fullStr The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title_full_unstemmed The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title_short The Clinical Aspect of Adaptor Molecules in T Cell Signaling: Lessons Learnt From Inborn Errors of Immunity
title_sort clinical aspect of adaptor molecules in t cell signaling: lessons learnt from inborn errors of immunity
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8397411/
https://www.ncbi.nlm.nih.gov/pubmed/34456914
http://dx.doi.org/10.3389/fimmu.2021.701704
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