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Table 1 The β -glucosidase activity of bone removed from patients with and without Gaucher disease (GD) before and after infusion with alglucerase

From: Failure of Alglucerase Infused into Gaucher Disease Patients to Localize in Marrow Macrophages

 

β-Glucosidase Activity (U/g DNA)

Chitotriosidase (U/g DNA)

 

Untreated

Treated

Expecteda

Normal Controls (4)

9.3 ± 5.3

—

—

10.0 ± 7.00

GD Patient 1

0.8

—

—

16

GD Patient 2

1.5

—

—

64

GD Patient 3

0.8

7.7b

>300

(48, 76)c

GD Patient 4

0.7

3.2d

>11.5

(110, 129)

GD Patient 5

1.5

2.6e

>600

(215, N/A)

GD Patient 6

—

2.0f

>4.7

(N/A, 70)

  1. Normal values are mean ± 1 standard deviation. N/A, not available.
  2. aExpected values are those that would be achieved if the infused enzyme were uniformly distributed to all body cells and remained with a t1/2 of 8 hr.
  3. bBone taken 8 hr after infusion of 60 U alglucerase/kg body wt.
  4. cValues in parentheses are pre- and postinfusion values, respectively.
  5. dBone taken at end of 2-hr infusion of 1.15 U alglucerase/kg body wt.
  6. eBone taken at end of 2-hr infusion of 60 U alglucerase/kg body wt.
  7. fBone taken 48 hr after infusion of 60 U alglucerase/kg body wt.
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Molecular Medicine

ISSN: 1528-3658