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Article

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.

Brain : a journal of neurology - 1 Apr 2013

Groh Janos, Kühl Thomas G, Ip Chi Wang, Nelvagal Hemanth R, Sri Sarmi, Duckett Steven, Mirza Myriam, Langmann Thomas, Cooper Jonathan D, Martini Rudolf

Abstract excerpt

The neuronal ceroid lipofuscinoses are fatal neurodegenerative disorders in which the visual system is affected early in disease progression. A typical accompanying feature is neuroinflammation, the pathogenic impact of which is presently obscure. Here we investigated the role of inflammatory cells in palmitoyl protein thioesterase 1-deficient (Ppt1(-/-)) mice, a model of infantile neuronal ceroid lipofuscinosis...

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PMID: 23485853
DOI: 10.1093/brain/awt020

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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