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Article

Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?

Biochemical Society transactions - 1 Oct 2014

Shyng Charles, Sands Mark S

Abstract excerpt

Infantile neuronal ceroid lipofuscinosis (INCL; infantile Batten disease) is an inherited paediatric neurodegenerative disease. INCL is caused by a deficiency in the lysosomal enzyme palmitoyl-protein thioesterase-1 (PPT1) and is thus classified as a lysosomal storage disease. Pathological examination of both human and murine INCL brains reveals progressive, widespread neuroinflammation. In fact, astrocyte...

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Source

Identifiers

PMID: 25233404
DOI: 10.1042/BST20140188

Genes

PPT1
GFAP

Provenance

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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