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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Egile Nagusiak:	Anu Jalanko, Jouni Vesa, Tuula Manninen, Carina von Schantz, Helena Minye, Anna-Liisa Fabritius, Tarja Salonen, Juhani Rapola, Massimiliano Gentile, Outi Kopra, Leena Peltonen
Formatua:	Artigo
Hizkuntza:	Inglês
Argitaratua:	Elsevier 2005-02-01
Saila:	Neurobiology of Disease
Gaiak:	INCL Inflammation Mouse model Neurodegeneration Palmitoyl protein thioesterase
Sarrera elektronikoa:	http://www.sciencedirect.com/science/article/pii/S0969996104001858
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