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

Journal of Human Genetics

Volumn 50, Issue 9, 2005, Pages 460-467

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

^a JAPAN SCIENCE AND TECHNOLOGY AGENCY   (Japan)

^b TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

^c UNIVERSITY OF TOKUSHIMA   (Japan)

^d YOKOHAMA CITY UNIVERSITY   (Japan)

Author keywords

Hexosaminidase; Enzyme replacement therapy; GM2 ganglioside; Lysosomal disease; Sandhoff disease; Schwann cell

Indexed keywords

4 METHYLUMBELLIFERYL N ACETYL BETA DEXTRO GLUCOSAMINE; 4 METHYLUMBELLIFERYL N ACETYL BETA DEXTRO GLUCOSAMINE 6 SULFATE; BETA HEXOSAMINIDASE S; BETA N ACETYLHEXOSAMINIDASE A; BETA N ACETYLHEXOSAMINIDASE B; CATION; GANGLIOSIDE GM2; GLUCOSAMINE DERIVATIVE; ISOENZYME; SOMATOMEDIN B RECEPTOR; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; BIOCHEMISTRY; CELL CULTURE; CELL IMMORTALIZATION; CHO CELL; CONTROLLED STUDY; DRUG DESIGN; ENZYME ACTIVITY; GENETIC TRAIT; HUMAN; HUMAN CELL; LIPID DEGRADATION; LIPID STORAGE; LYSOSOME; MOUSE; MOUSE STRAIN; NONHUMAN; PERIPHERAL NERVE; SANDHOFF DISEASE; SCHWANN CELL; SPINAL GANGLION;

ANIMALS; BETA-N-ACETYLHEXOSAMINIDASE; CELL CULTURE TECHNIQUES; CHO CELLS; CRICETINAE; CRICETULUS; FIBROBLASTS; GANGLIOSIDOSES, GM2; GENOTYPE; HUMANS; IMMUNOHISTOCHEMISTRY; LYSOSOMES; MICE; MICE, INBRED C57BL; MICROSCOPY, FLUORESCENCE; RECOMBINANT PROTEINS; SANDHOFF DISEASE; SCHWANN CELLS;

CRICETULUS GRISEUS;

EID: 26944444730     PISSN: 14345161     EISSN: None     Source Type: Journal    
DOI: 10.1007/s10038-005-0278-0     Document Type: Article

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