Mice with Ppt1Δex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons

Mice with Ppt1Δex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons

Infantile Neuronal Ceroid Lipofuscinosis (INCL) results from mutations in the palmitoyl protein thioesterase (PPT1, CLN1) gene and is characterized by dramatic death of cortical neurons. We generated Ppt1Δex4 mice by a targeted deletion of exon 4 of the mouse Ppt1 gene. Similar to the clinical pheno...

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Bibliographic Details
Main Authors: Anu Jalanko, Jouni Vesa, Tuula Manninen, Carina von Schantz, Helena Minye, Anna-Liisa Fabritius, Tarja Salonen, Juhani Rapola, Massimiliano Gentile, Outi Kopra, Leena Peltonen
Format: Article
Language:English
Published: Elsevier 2005-02-01
Series:Neurobiology of Disease
Subjects:
INCL
Inflammation
Mouse model
Neurodegeneration
Palmitoyl protein thioesterase
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996104001858

Internet

http://www.sciencedirect.com/science/article/pii/S0969996104001858

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