Article
Increased Expression of the Large Conductance, Calcium-Activated K+ (BK) Channel in Adult-Onset Neuronal Ceroid Lipofuscinosis.
PloS one - 1 Jan 2015
Donnelier Julien, Braun Samuel T, Dolzhanskaya Natalia, Ahrendt Eva, Braun Andrew P, Velinov Milen, Braun Janice E A
Abstract excerpt
Cysteine string protein (CSPα) is a presynaptic J protein co-chaperone that opposes neurodegeneration. Mutations in CSPα (i.e., Leu115 to Arg substitution or deletion (Δ) of Leu116) cause adult neuronal ceroid lipofuscinosis (ANCL), a dominantly inherited neurodegenerative disease. We have previously demonstrated that CSPα limits the expression of large conductance, calcium-activated K+ (BK) channels in neurons,...
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