Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis

Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis

The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited neurodegenerative diseases. Sheep affected with the CLN6 form provide a valuable model to investigate underlying disease mechanisms from preclinical stages. Excitatory neuron loss in these sheep is markedly regional, local...

Full description

Bibliographic Details
Main Authors: Manfred J. Oswald, David N. Palmer, Graham W. Kay, Karen J. Barwell, Jonathan D. Cooper
Format: Article
Language:English
Published: Elsevier 2008-10-01
Series:Neurobiology of Disease
Subjects:
Batten disease
Neuronal ceroid lipofuscinosis
CLN6
GABAergic interneurons
Ovine model
Lysosomal storage disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996108001307

Internet

http://www.sciencedirect.com/science/article/pii/S0969996108001307

Similar Items