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Blood, Vol. 107, Issue 10, 3959-3966, May 15, 2006
Leukocyte adhesion deficiency II patients with a dual defect of the GDP-fucose transporter
Blood Helmus et al.
107: 3959
Supplemental materials for: Helmus et al
Files in this Data Supplement:
- Figure S1. Quantitative comparison of AAL staining induced by wild-type and Δ22c GFTP (JPG, 47.5 JPG)
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Fibroblasts from an LAD II patient were transfected by nucleofection with empty vector (grey), wild-type GFTP (blue), and the  22C GFTP construct (red), respectively. The amount of DNA was titrated as indicated. Cell surface fucosylation was detected by AAL staining. The percentages of AAL-positive cells (region R; FL2-H between 3 × 101 and 3 × 102) are given. Mean fluorescence intensities of AAL staining of positive cells are shown in brackets.
- Figure S2. Titration of L-fucose used for rescue of fucosylation (JPG, 102 KB)
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Fibroblasts derived from patient IS were cultured with the indicated concentrations of L-fucose for 48 hrs before they were analyzed for fucosylation using AAL. AAL staining of untreated healthy fibroblasts is shown for comparison. Bar, 10 µm.
- Figure S3. Overexpression of wild-type GFTP but not of the GFTP of patient IS increases fucosylation under conditions of suboptimal fucose feeding (JPG, 87.4 KB)
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Fibroblasts were transfected with the indicated GFP-tagged GFTPs or control vector coding for GFP only. After 24 hrs, L-fucose was added to a concentration of 0.3 mM L-fucose. After further 24 hrs, cells were stained with anti-GFP and AAL. For comparison, a control vector-transfected culture grown without L-fucose is shown. Bar, 10 µm.
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