Journal
JOURNAL OF NEUROIMMUNOLOGY
Volume 361, Issue -, Pages -Publisher
ELSEVIER
DOI: 10.1016/j.jneuroim.2021.577745
Keywords
Fragile X syndrome; Astrocyte; Cortex; Purinergic signaling; Interleukin-6; STAT3; Tenascin C
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Funding
- McMaster University
- Brain Canada
- Azrieli Foundation [BC ANRP MIRI2013-3352]
- Canadian Institutes of Health Research Canada Graduate Scholarship (CIHR CGS-D)
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Symptoms of Fragile X syndrome (FXS) are partly driven by abnormal glial-mediated function, with elevated levels of immune-related factors interleukin-6 (IL-6) and tenascin C (TNC) released by FXS astrocytes. Purinergic signaling and immune regulatory pathways converge to promote pro-inflammatory responses in the FXS cortex.
The symptoms of Fragile X syndrome (FXS) are driven in part by abnormal glial-mediated function. FXS astro-cytes release elevated levels of immune-related factors interleukin-6 (IL-6) and tenascin C (TNC), and also demonstrate increased purinergic signaling, a pathway linked to signaling factor release. Here, in cortical as-trocytes from the Fmr1 knockout (KO) FXS mouse model, purinergic agonism enhanced TNC secretion and STAT3 phosphorylation, two processes linked to elevated IL-6 secretion in FXS, while STAT3 knockdown and TLR4 antagonism normalized Fmr1 KO IL-6 release. We therefore suggest that purinergic signaling and immune reg-ulatory pathways converge to drive FXS cortical pro-inflammatory responses.
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