ライフサイエンスコーパス: hexosaminidase A

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1 igosaccharide from GM2 was resistant to beta-hexosaminidase A.

2 HEXA and HEXB) encoding the subunits of beta-hexosaminidase A.

3 tion in the GM2-hydrolyzing activity of beta-hexosaminidase A.

4 ot the hydrolysis of GalNAc from GM2 by beta-hexosaminidase A.

5 conversion of ganglioside GM2 to GM3 by beta-hexosaminidase A.

6 ticipate in the formation of functional beta-hexosaminidase A activity as indicated by activator-depe

7 tive disorder caused by a deficiency of beta-hexosaminidase A activity.

8 for the heterodimeric lysosomal enzyme, beta-hexosaminidase A (alpha beta), as well as for the homodi

9 beta-Hexosaminidase A (alphabeta) is a heterodimer, whereas h

10 1-->4Glcbet a1-1'Cer) are refractory to beta-hexosaminidase A and sialidase, respectively, we have re

11 5Ac of 6'GM2 were readily hydrolyzed by beta-hexosaminidase A and sialidase, respectively, without GM

12 showed significant co-localization with beta-hexosaminidase A and the azurophilic marker MPO in human

13 Accumulation of beta-hexosaminidases A and B substrates is presumed to cause

14 sulfated substrates were highly specific for hexosaminidase A, and in fractionated serum, cells, and

15 responsible for the metabolic bypass of beta-hexosaminidase A deficiency.

16 ssed human azurophilic granule-resident beta-hexosaminidase A displayed the capacity to generate pauc

17 es and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-d-galactosamin

18 ity comparable with that of recombinant beta-hexosaminidase A formed by the co-expression of the alph

19 n to stimulate the hydrolysis of GM2 by beta-hexosaminidase A, GM2 activator was found to bind avidly

20 , various strategies aimed at restoring beta-hexosaminidase A have been explored.

21 prevents the formation of a functional beta-hexosaminidase A heterodimer resulting in the severe neu

22 the brain, is caused by a deficiency of beta-hexosaminidase A (Hex A) or GM2 activator.

23 atabolism of GM2 to GM3 in man requires beta-hexosaminidase A (HexA) and a protein cofactor, the GM2

24 Loss of function of the enzyme beta-hexosaminidase A (HexA) causes the lysosomal storage dis

25 eted high levels of biologically active beta-hexosaminidase A in vitro and cross-corrected the metabo

26 bstrate (GM2) for the defective enzyme (beta-hexosaminidase A) prevents GSL accumulation and the neur

27 complex, which interacts with the hydrolase Hexosaminidase A, the enzyme that cleaves the terminal s

28 Among human isozymes, only beta-hexosaminidase A together with the GM2 activator protein

29 The % hexosaminidase A values as derived from the ratio betwee

30 seases that are caused by deficiency of beta-hexosaminidase A, which comprises an alphabeta heterodim

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