Introduction of an N-glycan sequon into HEXA enhances human beta-hexosaminidase cellular uptake in a model of Sandhoff disease.

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2010 nî lūn-bûn

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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name

Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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P1476

Introduction of an N-glycan se ...... n a model of Sandhoff disease.

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P2093

Daisuke Tsuji

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Fumiko Matsuzawa

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Hitoshi Sakuraba

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Kazuhiko Matsuoka

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Kohji Itoh

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Sei-Ichi Aikawa

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P2860

AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2

Gene encoding the human beta-hexosaminidase beta chain: extensive homology of intron placement in the alpha- and beta-chain genes

Analysis of the glycosylation and phosphorylation of the alpha-subunit of the lysosomal enzyme, beta-hexosaminidase A, by site-directed mutagenesis

Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease

Insulin-like growth factor-II/mannose-6-phosphate receptor: widespread distribution in neurons of the central nervous system including those expressing cholinergic phenotype

Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis

The biogenesis of lysosomes

Intrathecal enzyme replacement therapy: successful treatment of brain disease via the cerebrospinal fluid

Inefficiency in GM2 ganglioside elimination by human lysosomal beta-hexosaminidase beta-subunit gene transfer to fibroblastic cell line derived from Sandhoff disease model mice.

Production of recombinant beta-hexosaminidase A, a potential enzyme for replacement therapy for Tay-Sachs and Sandhoff diseases, in the methylotrophic yeast Ogataea minuta

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scholarly article

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10.1038/MT.2010.113

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2010-06-22T00:00:00Z

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44695899

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20571546

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