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. Surprisi...

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Autors principals: Kielar, Catherine, Maddox, Lucy, Bible, Ellen, Pontikis, Charlie C, Macauley, Shannon L, Griffey, Megan A, Wong, Michael, Sands, Mark S, Cooper, Jonathan D
Format: Artigo
Idioma:Inglês
Publicat: 2006
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC1866219/
https://ncbi.nlm.nih.gov/pubmed/17046272
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2006.09.001
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