Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis

Infantile neuronal ceroid lipofuscinosis (INCL) is caused by deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1 (PPT1). We have investigated the onset and progression of pathological changes in Ppt1 deficient mice (Ppt1−/−) and the development of their seizure phenotype. Surprising...

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Bibliographic Details
Main Authors: Catherine Kielar, Lucy Maddox, Ellen Bible, Charlie C. Pontikis, Shannon L. Macauley, Megan A. Griffey, Michael Wong, Mark S. Sands, Jonathan D. Cooper
Format: Article
Language:English
Published: Elsevier 2007-01-01
Series:Neurobiology of Disease
Subjects:
Infantile neuronal ceroid lipofuscinosis
Batten disease
PPT1
Thalamic neurodegeneration
Seizures
GABAergic interneurons
Online Access:http://www.sciencedirect.com/science/article/pii/S096999610600221X

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http://www.sciencedirect.com/science/article/pii/S096999610600221X

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