Topic: P2X4 receptor

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a selective loss of motor neurons (MN). During ALS, ATP released by neurons and glial cells modulates the neuroglial interplay via activation of P2X receptors. Among these, P2X4 receptor, which is a non-selective cationic channel, has been recently involved in ALS pathogenesis. We found that both the absence of P2X4 or the increase of surface P2X4 using P2X4KO or non-internalized P2X4KI mice respectively, have a beneficial outcome in the SOD1 mice. This paradoxical output suggests a complex cell-role of P2X4 in

P2X receptors are ligand-gated cation channels that play key roles in the central nervous system (CNS). Among those, P2X4 receptor, the most abundant P2X receptors expressed in the brain including neurons and microglia, are constitutively internalized and retained mainly into the lysosomes with a weak surface expression in normal conditions. Microglia functions have been directly linked to the regulation of lysosomal activity, which contributes to the brain homeostasis. Interestingly, P2X4 preserve their functionality into lysosomes and their activation by intraluminal ATP allows calcium influ