Expression of Palmitoyl Protein Thioesterase in Neurons

• Published in: Molecular genetics and metabolism Vol. 69; no. 2; pp. 123 - 129
• Main Authors: Heinonen, Outi, Kyttälä, Aija, Lehmus, Elina, Paunio, Tiina, Peltonen, Leena, Jalanko, Anu
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.02.2000
• Subjects: Adenoviridae - genetics; Adenovirus vectors; Animals; brain; Brain - enzymology; Cells, Cultured; Genetic Vectors; Immunohistochemistry; infantile neuronal ceroid lipofuscinoses; Mice; Mice, Inbred Strains; Neurons - cytology; Neurons - enzymology; palmitoyl protein thioesterase; PC12 Cells; primary neurons; Rats; Recombination, Genetic; Thiolester Hydrolases - metabolism; primary neurons; Adenovirus vectors; PC12-cells; infantile neuronal ceroid lipofuscinoses; brain; palmitoyl protein thioesterase

Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder in childhood that is caused by mutations in the gene encoding lysosomal palmitoyl protein thioesterase (PPT). INCL is characterized by massive and selective loss of cortical neurons. Here we have analyzed the intr...