Document Detail

Establishment of immortalized Schwann cells from Sandhoff mice and corrective effect of recombinant human beta-hexosaminidase A on the accumulated GM2 ganglioside.
MedLine Citation:
PMID:  16180049     Owner:  NLM     Status:  MEDLINE    
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 (beta-hexosaminidases A and S) were decreased, and GM2 ganglioside accumulated in lysosomes of the cells. Incorporation of recombinant human beta-hexosaminidase isozymes expressed in Chinese hamster ovary cells into the cultured Sandhoff Schwann cells via cation-independent mannose 6-phosphate receptors was found, and the incorporated beta-hexosaminidase A degraded the accumulated GM2 ganglioside. The established Sandhoff Schwann cell line is useful for investigation and development of therapies for Sandhoff disease.
Mai Ohsawa; Masaharu Kotani; Youichi Tajima; Daisuke Tsuji; Yasuhiro Ishibashi; Aya Kuroki; Kohji Itoh; Kazuhiko Watabe; Kazunori Sango; Shoji Yamanaka; Hitoshi Sakuraba
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2005-09-23
Journal Detail:
Title:  Journal of human genetics     Volume:  50     ISSN:  1434-5161     ISO Abbreviation:  J. Hum. Genet.     Publication Date:  2005  
Date Detail:
Created Date:  2005-10-18     Completed Date:  2006-05-02     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9808008     Medline TA:  J Hum Genet     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  460-7     Citation Subset:  IM    
CREST, JST, Kawaguchi, Japan.
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MeSH Terms
CHO Cells
Cell Culture Techniques / methods*
Gangliosidoses, GM2 / metabolism*
Lysosomes / metabolism
Mice, Inbred C57BL
Microscopy, Fluorescence
Recombinant Proteins / pharmacology*
Sandhoff Disease / enzymology*
Schwann Cells / cytology*,  drug effects,  enzymology
beta-N-Acetylhexosaminidases / pharmacology*
Reg. No./Substance:
0/Recombinant Proteins; EC

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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