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Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis.

Cell stem cell - 4 Sept 2009

Tamaki Stanley J, Jacobs Yakop, Dohse Monika, Capela Alexandra, Cooper Jonathan D, Reitsma Michael, He Dongping, Tushinski Robert, Belichenko Pavel V, Salehi Ahmad, Mobley William, Gage Fred H, Huhn Stephen, Tsukamoto Ann S, Weissman Irving L, Uchida Nobuko

Abstract excerpt

Infantile neuronal ceroid lipofuscinosis (INCL) is a fatal neurodegenerative disease caused by a deficiency in the lysosomal enzyme palmitoyl protein thioesterase-1 (PPT1). Ppt1 knockout mice display hallmarks of INCL and mimic the human pathology: accumulation of lipofuscin, degeneration of CNS neurons, and a shortened life span. Purified non-genetically modified human CNS stem cells, grown as neurospheres...

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Identifiers

PMID: 19733542
DOI: 10.1016/j.stem.2009.05.022

Genes

PPT1

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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