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Table 2 HexA activity of the study group

From: Effect of cyclic, low dose pyrimethamine treatment in patients with Late Onset Tay Sachs: an open label, extended pilot study

Patient

Baseline activity nmol/hour/mg protein

Baseline HexA activity, (% of wild type*)

Peak HexA activity (% of wild type*)

Peak increase in HexA activity (fold Increase) relative to to baseline

1

92.1

1.3

2.5

1.87

2

84.8

1.2

2.5

2.1

3

63.3

0.9

1.8

2

4

48.3

0.7

2.1

3

  1. HexA activity in lymphocyte was determined using MUGS as an artificial substrate as described in the Methods. [% of wild type*] refers to mean activity in lymphocyte preparation from unaffected healthy subjects (“wild type”): 6900 ± 996 nmol/mg protein/hour (n = 3). Relative change in activity refers to fold change relative to baseline, where baseline activity or activity unchanged from baseline is referred to as 1 (no change), 2 fold describes doubling, 3 fold refers to tripling, etc.).
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172