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

Abstract：

: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 Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profiling showed deregulation of key neuronal functions in knock-out mice including cholesterol metabolism, neuronal maturation, and calcium homeostasis. Cholesterol metabolism showed major changes; sterol biosynthesis was enhanced and steady-state amounts of sterols were altered at the cellular level. Changes were also present in early maturation of Ppt1(Deltaex4) neurons indicated by increased proliferative capacity of neuronal stem cells. Knock-out neurons presented unaltered electrophysiological properties suggesting uncompromised synaptic function in young animals. However, knock-out neurons exhibited more efficient recovery from glutamate-induced calcium transients, possibly indicating neuroprotective activation. This study established that the neuronal deregulation in INCL is linked to neuronal maturation, lipid metabolism and calcium homeostasis.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Ahtiainen L,Kolikova J,Mutka AL,Luiro K,Gentile M,Ikonen E,Khiroug L,Jalanko A,Kopra O

doi

10.1016/j.nbd.2007.06.012

subject

Has Abstract

pub_date

2007-10-01 00:00:00

pages

52-64

issue

1

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(07)00133-7

journal_volume

28

pub_type

杂志文章

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