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 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.
Department of Neurodegenerative Disease, Institute of Neurology, University College London, London, UK.
Ulrike
U
Träger
Department of Experimental Medical Science, Wallenberg Neuroscience Center, Lund University, Lund, Sweden.
Anna
A
Magnusson
University College London, London, UK.
Nayana
N
Lahiri Swales
Institute of Neurology, University College London, London, UK; Bristol University, Bristol UK.
Edward
E
Wild
University College London, London, UK.
Janet
J
North
University College London, London, UK.
Mark
M
Lowdell
Department of Experimental Medical Science, Wallenberg Neuroscience Center, Lund University, Lund, Sweden.
Maria
M
Björkqvist