Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis.
Mutations in the CLN6 gene cause a variant late infantile form of neuronal ceroid lipofuscinosis (NCL; Batten disease).CLN6 loss leads to disease clinically characterized by vision impairment, motor and cognitive dysfunction, and seizures.Accumulating evidence suggests that alterations in metal homeostasis and cellular signaling pathways are implic