Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro‐inflammatory phenotype

Juvenile neuronal ceroid lipofuscinosis (JNCL) is a lysosomal storage disease caused by an autosomal recessive mutation in CLN3. Regions of microglial activation precede and predict areas of neuronal loss in JNCL; however, the functional role of activated microglia remains to be defined. The inflamm...

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Veröffentlicht in:	Journal of neurochemistry 2013-10, Vol.127 (2), p.245-258
Hauptverfasser:	Xiong, Juan, Kielian, Tammy
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Apoptosis Bone Marrow Cells - physiology Brain Caspase 1 - metabolism caspase‐1 Cells, Cultured Chemokines - metabolism CLN3 Cytokines - metabolism Cytotoxicity Electron Spin Resonance Spectroscopy Enzyme-Linked Immunosorbent Assay Glutamic Acid - metabolism Humans IL‐1β inflammasome Inflammation - pathology juvenile Batten disease Macrophages - physiology Membrane Glycoproteins - genetics Mice Mice, Inbred C57BL Mice, Knockout microglia Microglia - pathology Microscopy, Confocal Molecular Chaperones - genetics Neurochemistry Neurodegeneration Neuronal Ceroid-Lipofuscinoses - pathology Neurons Phenotype Primary Cell Culture
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