Pdf characterization of a gdpfucose transporter and a. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. Acts as the major nucleotidesugar transporter for the import of gdp fucose into the golgi lumen. Slc35c1 gdpfucose transporter 1 homo sapiens human. The identification of a novel gdp fucose transporter and characterization of its function and intracellular distribution might help to. We have 715 the transporter movie torrents for you. May play an important role in the cellular response to tissue hypoxia. Congenital disorder of glycosylation type iic wikimili. Similar results were observed on fucose therapy in fucose transporter. A second lad syndrome, ladii, is caused by inherited defects in the gene encoding guanosine diphosphate gdp fucose transporter 1, which is a key player in fucose metabolism. The gene encoding atpbinding cassette transporter 1 is mutated in tangier disease. This message will disappear when all data is loaded.
Nucleotide sugar transporters of the golgi apparatus. Lad 2 is caused by mutations in the gene which encodes the gdp fucose transporter 1 fuct1. In eight patients with slc35a2cdg in the literature table 1, manifestations include. Golgi gdpfucose transporterdeficient mice mimic congenital.
Structure and function of nucleotide sugar transporters. The arabidopsis golgilocalized gdplfucose transporter. Slc35c1 antibody lsc345427 is an unconjugated rabbit polyclonal antibody to slc35c1 cterminus from human and rat. Gdp fuc and not gdp man, and our in vivo analysis supports its role as the primary golgi gdp fuc transporter. Here we identify gdp fucose transporter 1 gft1, an arabidopsis nucleotide sugar. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists. Gdp fucose transporter 1 468 words exact match in snippet view article find links to article gdp fucose transporter 1 is a protein that in humans is encoded by the slc35c1 gene. Omim entry 609182 solute carrier family 35, member. Defects can be associated with congenital disorder of glycosylation type iic. Nova lifetech human gdp fucose transporter 1, slc35c1 elisa kit sku. Due to this newly attributed function, we have revised its name to gdp fucose transporter 1 gft1. Phylogenetic tree of gdp l fucose and gdp dmannose transporters from eukaryotes based on rautengarten et al.
Transports gdp fucose in a strict counterexchange mode. A high expression of gdp fucose transporter in hepatocellular carcinoma is a key factor for increases in. Indeed, glycosylation analysis of endogenous proteins demonstrated the presence of paucimannosidic glycans bearing an. Altered expression of fucosylation pathway genes is. Gdp fucose is transported into the golgi lumen by gdp fucose transporter 1 slc35c1. A blastp search was carried out in the sequence data of p. Defects can be associated with congenital disorder of glycosylation. By searching for sequences similar to gdp fucose transporter 1 slc35c1. Mammalian cells that do not transport gdp mannose into the golgi were used to demonstrate that leishmania lpg2 is a gdp mannose transporter that can also transport gdp arabinose and gdp fucose. A fucose residue from gdp fucose is transferred to the sugar moieties of glycoconjugates or the serine. Wikigenes slc35c1 solute carrier family 35 gdpfucose. Order nova lifetechs human gdp fucose transporter 1.
The arabidopsis golgilocalized gdp l fucose transporter. The annotation score provides a heuristic measure of the annotation content of a uniprotkb entry or. Involved in gdpfucose import from the cytoplasm into the golgi lumen. Congenital disorder of glycosylation type iic or leukocyte adhesion deficiency2 lad2 is a type of leukocyte adhesion deficiency attributable to the absence of neutrophil sialyllewisx, a ligand of p and eselectin on vascular endothelium. Using proteoliposomebased transport assays, we show that gft preferentially transports gdp l fucose over other nucleotide sugars in vitro, while gft1 silenced plants are almost devoid of l fucose in cell wallderived xyloglucan and rhamnogalacturonan ii.
The first indication that gdp fucose, like other nucleotide sugars, is transported by a carriermediated process was obtained by sommers and hirschberg 1982. Here we identify gdpfucose transporter 1 gft1, an arabidopsis nucleotide sugar transporter that translocates gdplfucose into the golgi lumen. Golgi gdpfucose transporterdeficient mice mimic congenital disorder of glycosylation iicleukocyte adhesion deficiency ii. The rcsb pdb also provides a variety of tools and resources. Slc35c1 antibody lsc445895 is an hrpconjugated rabbit polyclonal antibody to slc35c1 cterminus from human and rat. As a member of the wwpdb, the rcsb pdb curates and annotates pdb data according to agreed upon standards.
The gene defective in leukocyte adhesion deficiency ii encodes a putative gdpfucose transporter. The gene defective in leukocyte adhesion deficiency ii encodes a. Adhesion deficiency ii encodes a putative gdpfucose transporter. Inactivation of gdpfucose transporter gene slc35c1 in. This test analyzes up to 27 genes that are associated with hereditary phagocyte defects hpd. Frontiers characterization of a gdpfucose transporter. Does not affect subcellular localization to the golgi apparatus. Gdpfucose transporter 1 is a protein that in humans is encoded by the slc35c1 gene. It aims to collect, preserve, and showcase the intellectual output of staff and students of the university of melbourne for a global audience. Wikiproject molecular and cell biology rated stubclass, lowimportance this article is. Is required for proper plant growth and development pubmed. The deduced 337amino acid protein has a calculated molecular mass of 36. Shows lack of both core alpha 1,3 and alpha 1,6linked fucose residues on nglycans. Antislc35c1 antibody rabbit antihuman polyclonal wb.
Also provided are yeast cells which express a gdp l fucose transporter andor a fucosyl transferase with the bifunctional enzyme. A yeast strain expressing a bifunctional fucokinase gdp l fucose pyrophosphorylase enzyme and capable of producing gdp l fucose in vivo is provided. Complementation cloning identifies cdgiic, a new type of congenital disorders of glycosylation, as a gdp fucose transporter. Gdp fucose transporter 1 literature citations complementation cloning identifies cdgiic, a new type of congenital disorders of glycosylation, as a gdp fucose transporter deficiency. The gdp fucose transporter 1 defect results in the absence of sialyl lewis x and other structurally related fucosylated glycans, which make up the carbohydrate ligands. Minerva access is the universitys institutional repository. Gdp fucose transporter 1, solute carrier family 35, member c1, multispan transmembrane protein fuct1, gdp fucose transporter, gft, fc03b12, solute carrier family 35 member c1, gdp fucose transporter 1 like, golgi gdp fucose translocator. Es2362419t3 cells with depression or deletion of protein.
Slc35c1 gene genecards fuct1 protein fuct1 antibody. Slc35 family of nucleotide sugar transporters in the iupharbps guide to pharmacology. Using proteoliposomebased transport assays, we show that gft preferentially transports gdplfucose over other nucleotide sugars in vitro, while gft1silenced plants are almost devoid of lfucose in cell wallderived xyloglucan and rhamnogalacturonan ii. Antislc35c1 antibody rabbit antihuman polyclonal hrp. May be either a gdp fucose transporter that competes with slc35c1 for gdp fucose, or a factor that otherwise enhances the fucosylation of notch and is required for optimal notch signaling in mammalian cells.
Cdg2c, flj120, fuct1, gdp fucose transporter 1, solute carrier family 35 member c1. In addition, the said yeast contains one or more expression cassettes for fusion proteins of heterologous. This leads to an absence of fucosylated carbohydrate ligands which bind selectins on the endothelium resulting in defective leukocyte rolling. Omim entry 605881 solute carrier family 35, member. The gene defective in leukocyte adhesion deficiency ii encodes a putative gdp fucose transporter. Full images of the western blots used in the present study. Another initiation cofactor, if2b, removes gdp from. Acts also as a gdp mannose transporter that may be involved in the import of gdp mannose from the cytoplasm into the golgi lumen pubmed. Here we identify gdp fucose transporter 1 gft1, an arabidopsis nucleotide sugar transporter that translocates gdp l fucose into the golgi lumen. In 2 individuals with congenital disorder of glycosylation type iic cdg2c. Disease description a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by underglycosylated serum glycoproteins.
The gene encoding the gdp fucose transporter was identified independently by two groups using complementation cloning in patientderived cells lubke et al. Discovery of the islet hormones produced a torrent of studies on. Genetic testing of these genes may confirm a diagnosis and help guide treatment and management decisions. Two pathways for importing gdpfucose into the endoplasmic. Nucleotide sugar transport across golgi membranes is essential for the luminal biosynthesis of glycan structures. These results suggested the existence of one or more additional gdp fucose transporter genes in drosophila and mammals.
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