Figure 2.
Urinary oligosaccharides and human PPCA expression in GS mice after BMT. (A) Cathepsin A activities in tissues of BMT mice. GS mice, transplanted with either wild-type or transgenic (T24) BM at 2.5 months of age were sacrificed 2 months later, and tissue lysates were assayed for cathepsin A activity. (B) Urine samples were collected from 1-month-old wild-type and GS mice before BMT (lanes B) and 1 month after BMT (lanes A). Mouse GS1 and GS2 received wild-type and transgenic BM, respectively. A molecular weight ladder of glucose polymers (M), with the (Glucose)4 for reference, is shown. Oligosaccharides larger than this tetrasaccharide are abnormal. (C) Western blot of tissues from BMT mice were probed with α32 antibodies: GS1 and GS2 mice received transgenic BM (BM-T24) and GS3 mouse received wild-type BM (BM-WT). The exposure time for the brain was 12 times longer than that for the other tissues.