Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis

Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis

Infantile neuronal ceroid lipofuscinosis (INCL) is one of a group of fatal hereditary lysosomal storage disorders. Palmitoyl protein thioesterase 1 null mutant mice (PPT1−/−) now exist that accurately recapitulate many important disease features. The severely affected PPT1−/− mouse CNS exhibited red...

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Bibliographic Details
Main Authors: Ellen Bible, Praveena Gupta, Sandra L Hofmann, Jonathan D Cooper
Format: Article
Language:English
Published: Elsevier 2004-07-01
Series:Neurobiology of Disease
Subjects:
Neuronal ceroid lipofuscinosis
CLN1
PPT1
INCL
GABAergic interneurons
Glial activation
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996104000440

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http://www.sciencedirect.com/science/article/pii/S0969996104000440

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