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Article

Establishment of immortalized Schwann cells from Sandhoff mice and corrective effect of recombinant human beta-hexosaminidase A on the accumulated GM2 ganglioside.

Journal of human genetics - 1 Jan 2005

Ohsawa Mai, Kotani Masaharu, Tajima Youichi, Tsuji Daisuke, Ishibashi Yasuhiro, Kuroki Aya, Itoh Kohji, Watabe Kazuhiko, Sango Kazunori, Yamanaka Shoji, Sakuraba Hitoshi

Abstract excerpt

We have established spontaneously immortalized Schwann cell lines from dorsal root ganglia and peripheral nerves of Sandhoff mice. One of the cell lines exhibited genetically and biochemically distinct features of Sandhoff Schwann cells. The enzyme activities toward 4-methylumbelliferyl N-acetyl-beta-D-glucosamine (beta-hexosaminidases A, B, and S) and 4-methylumbelliferyl N-acetyl-beta-D-glucosamine-6-sulfate...

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PMID: 16180049
DOI: 10.1007/s10038-005-0278-0

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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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