International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience
-
Int. J. Dev. Neurosci. · Jun 1994
Growth factor-induced neurite growth in primary neuronal cultures of dogs with neuronal ceroid lipofuscinosis.
Neuronal ceroid lipofuscinosis (NCL) is a type of lysosomal storage disease resulting in the progressive deterioration of neuronal function. Little is known about the genetics, pathophysiology and biochemical basis of this disease. This is, in part, due to the complexity of the central nervous system and the lack of an in vitro model. ⋯ We report the establishment and morphological characterization of neuronal cultures from normal and NCL dog brains. The abnormal morphology of cultured NCL neurons can, in part, be alleviated by supplementing the medium with growth factors. The results suggest that this cellular model of NCL will be useful to study the molecular and physiological mechanisms of NCL disease, as well as to test potential therapeutic agents and candidate genes.