JAK/STAT Signalling in Huntington’s Disease Immune Cells
Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. Both central and peripheral innate immune activation have been described as features of the disease. Isolated human HD monocytes have been shown to produce more cytokin...
Main Authors: | , , , , , , |
---|---|
Format: | Online |
Language: | English |
Published: |
Public Library of Science
2013
|
Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3871417/ |
id |
pubmed-3871417 |
---|---|
recordtype |
oai_dc |
spelling |
pubmed-38714172013-12-26 JAK/STAT Signalling in Huntington’s Disease Immune Cells Träger, Ulrike Magnusson, Anna Lahiri Swales, Nayana Wild, Edward North, Janet Lowdell, Mark Björkqvist, Maria Pathogenic Mechanism Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. Both central and peripheral innate immune activation have been described as features of the disease. Isolated human HD monocytes have been shown to produce more cytokines upon LPS stimulation compared to control monocytes. Understanding alterations in the signalling cascades responsible and activated by this increase in pro-inflammatory cytokine production is crucial in understanding the molecular basis of this phenomenon. Here we investigated the signalling cascade most commonly activated by pro-inflammatory cytokines such as IL-6 – the JAK/STAT signalling cascade. Using flow cytometry, we show that one out of three key transcription factors activated by JAK/STAT signalling is altered in primary human HD innate immune cells, suggesting that this pathway may only play a minor, additive role in the immune cell dysfunction in HD. Public Library of Science 2013-12-13 /pmc/articles/PMC3871417/ /pubmed/24459609 http://dx.doi.org/10.1371/currents.hd.5791c897b5c3bebeed93b1d1da0c0648 Text en http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited. |
repository_type |
Open Access Journal |
institution_category |
Foreign Institution |
institution |
US National Center for Biotechnology Information |
building |
NCBI PubMed |
collection |
Online Access |
language |
English |
format |
Online |
author |
Träger, Ulrike Magnusson, Anna Lahiri Swales, Nayana Wild, Edward North, Janet Lowdell, Mark Björkqvist, Maria |
spellingShingle |
Träger, Ulrike Magnusson, Anna Lahiri Swales, Nayana Wild, Edward North, Janet Lowdell, Mark Björkqvist, Maria JAK/STAT Signalling in Huntington’s Disease Immune Cells |
author_facet |
Träger, Ulrike Magnusson, Anna Lahiri Swales, Nayana Wild, Edward North, Janet Lowdell, Mark Björkqvist, Maria |
author_sort |
Träger, Ulrike |
title |
JAK/STAT Signalling in Huntington’s Disease Immune Cells |
title_short |
JAK/STAT Signalling in Huntington’s Disease Immune Cells |
title_full |
JAK/STAT Signalling in Huntington’s Disease Immune Cells |
title_fullStr |
JAK/STAT Signalling in Huntington’s Disease Immune Cells |
title_full_unstemmed |
JAK/STAT Signalling in Huntington’s Disease Immune Cells |
title_sort |
jak/stat signalling in huntington’s disease immune cells |
description |
Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. Both central and peripheral innate immune activation have been described as features of the disease. Isolated human HD monocytes have been shown to produce more cytokines upon LPS stimulation compared to control monocytes. Understanding alterations in the signalling cascades responsible and activated by this increase in pro-inflammatory cytokine production is crucial in understanding the molecular basis of this phenomenon. Here we investigated the signalling cascade most commonly activated by pro-inflammatory cytokines such as IL-6 – the JAK/STAT signalling cascade. Using flow cytometry, we show that one out of three key transcription factors activated by JAK/STAT signalling is altered in primary human HD innate immune cells, suggesting that this pathway may only play a minor, additive role in the immune cell dysfunction in HD. |
publisher |
Public Library of Science |
publishDate |
2013 |
url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3871417/ |
_version_ |
1612040921175556096 |