Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction

Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction

Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1Deltaex4 knock-out mouse neurons in culture and acute brain slices. Global...

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Bibliographic Details
Main Authors: Laura Ahtiainen, Julia Kolikova, Aino-Liisa Mutka, Kaisu Luiro, Massimiliano Gentile, Elina Ikonen, Leonard Khiroug, Anu Jalanko, Outi Kopra
Format: Article
Language:English
Published: Elsevier 2007-10-01
Series:Neurobiology of Disease
Subjects:
Palmitoyl protein thioesterase 1
Infantile neuronal ceroid lipofuscinosis
Neuron culture
Synapse
Neuron maturation
Calcium homeostasis
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996107001337

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http://www.sciencedirect.com/science/article/pii/S0969996107001337

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