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...

תיאור מלא

מידע ביבליוגרפי

Principais autores:	Anu Jalanko, Jouni Vesa, Tuula Manninen, Carina von Schantz, Helena Minye, Anna-Liisa Fabritius, Tarja Salonen, Juhani Rapola, Massimiliano Gentile, Outi Kopra, Leena Peltonen
פורמט:	Artigo
שפה:	Inglês
יצא לאור:	Elsevier 2005-02-01
סדרה:	Neurobiology of Disease
נושאים:	INCL Inflammation Mouse model Neurodegeneration Palmitoyl protein thioesterase
גישה מקוונת:	http://www.sciencedirect.com/science/article/pii/S0969996104001858
תגים:	הוספת תג אין תגיות, היה/י הראשונ/ה לתייג את הרשומה!