Rescue in fucosylation of LADII fibroblasts by complementation with the full-length GDP-fucose transporter gene.

Skin fibroblasts obtained from the patient were transfected with a bicistronic vector containing a GFP reporter gene and the GDP-fucose transporter cDNA obtained from healthy (A-B) or LADII cells (C-D). Five days after transfection, LADII fibroblasts were stained with AAL to assess fucosylation (red). (A) Fibroblasts transfected (GFP-positive; green) with wild-type GDP-fucose transporter exhibit strong perinuclear staining, whereas (C) cells transfected with the ΔG588 transporter gene did not stain with AAL. The lower panels (B-D) show Nomarski optic images of the same fields as in panels A and C. Scale bar is approximately 50 μm.