作者: Kerstin Lühn , Anna Laskowska , Jan Pielage , Christian Klämbt , Ute Ipe
DOI: 10.1016/J.YEXCR.2004.08.043
关键词:
摘要: Nucleotide sugar transporters play a central role in the process of glycosylation. They are responsible for translocation nucleotide sugars from cytosol, their site synthesis, into Golgi apparatus where activated serve as substrates variety glycosyltransferases. We and others have recently identified cloned first GDP-fucose H. sapiens C. elegans. Based on sequence similarity, we could identify putative homolog Drosophila melanogaster showing about 45% identity protein level. The gene (CG9620) encodes highly hydrophobic, multi-transmembrane spanning 38.1 kDa that is localized apparatus. In order to test whether this serves transporter, performed complementation studies with fibroblasts patient LADII (leukocyte adhesion deficiency II) which exhibit strong reduction fucosylation due point mutation human transporter gene. show transient transfection these cells CG9620 cDNA corrects transport defect reestablishes fucosylation. This study gives experimental proof product silico functional transporter.