Influence of N-acetylhomocysteine thiolactone on cultured retinal cells in canine neuronal lipofuscinosis. An ultrastructural study.
An SH- group donor drug, N-acetylhomocysteine thiolactone (0.2 mg/ml), was added to retinal cultures from an adult dog affected with ceroid lipofuscinosis (NCL). This substance was applied every 3rd day to the culture medium. After 1, 2 and 3 weeks in culture the cells were fixed for electron microscopy. Ultrastructurally the NCL-specific lipopigments revealed extensive fading out or vacuolation, revealing randomly arranged lamellar or 'fingerprint' membranes as well as structures resembling degenerated mitochondria. Additionally extensive fragmentation of the pigment bodies was present. The significance of these results are discussed.
D000818:Animals; D002478:Cells, Cultured; D004283:Dog Diseases / Q000473:pathology*; D004285:Dogs; D006801:Humans; D008854:Microscopy, Electron; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology / Q000662:veterinary*; D012160:Retina / Q000187:drug effects / Q000648:ultrastructure*; D013876:Thiophenes / Q000494:pharmacology*
D013876:Thiophenes; C000486:citiolone