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The Role of Attenuated Astrocyte Activation in Infantile Neuronal Ceroid Lipofuscinosis

26 Oct 2011

Abstract excerpt

Infantile neuronal ceroid lipofuscinosis (INCL) is an inherited neurodegenerative disorder affecting the CNS during infancy. INCL is caused by mutations in the CLN1 gene that lead to a deficiency in the lysosomal hydrolase, palmitoyl protein thioesterase 1 (PPT1). A murine model of INCL, the PPT1-deficient (PPT1(-/-)) mouse, is an accurate phenocopy of the human disease. The first pathological change observed in...

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PMID: 22031903
DOI: 10.1523/jneurosci.3579-11.2011

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