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...

Ausführliche Beschreibung

Bibliografische Detailangaben

Hauptverfasser:	Laura Ahtiainen, Julia Kolikova, Aino-Liisa Mutka, Kaisu Luiro, Massimiliano Gentile, Elina Ikonen, Leonard Khiroug, Anu Jalanko, Outi Kopra
Format:	Artigo
Sprache:	Inglês
Veröffentlicht:	Elsevier 2007-10-01
Schriftenreihe:	Neurobiology of Disease
Schlagworte:	Palmitoyl protein thioesterase 1 Infantile neuronal ceroid lipofuscinosis Neuron culture Synapse Neuron maturation Calcium homeostasis
Online-Zugang:	http://www.sciencedirect.com/science/article/pii/S0969996107001337
Tags:	Tag hinzufügen Keine Tags, Fügen Sie das erste Tag hinzu!