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Article

Impaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff disease.

PloS one - 1 Jan 2013

Ogawa Yasuhiro, Tanaka Makoto, Tanabe Miho, Suzuki Toshihiro, Togawa Tadayasu, Fukushige Tomoko, Kanekura Takuro, Sakuraba Hitoshi, Oishi Kazuhiko

Abstract excerpt

Sandhoff disease (SD) is a glycosphingolipid storage disease that arises from mutations in the Hexb gene and the resultant deficiency in β-hexosaminidase activity. This deficiency results in aberrant lysosomal accumulation of the ganglioside GM2 and related glycolipids, and progressive deterioration of the central nervous system. Dysfunctional glycolipid storage causes severe neurodegeneration through a poorly...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 23383290
DOI: 10.1371/journal.pone.0055856
PMCID: PMC3561340

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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