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http://purl.uniprot.org/citations/15653671http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/15653671http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/15653671http://www.w3.org/2000/01/rdf-schema#comment"LADII (leukocyte adhesion deficiency type II)/CDGIIc (congenital disorder of glycosylation type IIc) is a rare autosomal recessive disease characterized by leukocyte adhesion deficiency as well as severe neurological and developmental abnormalities. It is caused by mutations in the Golgi GDP-fucose transporter, resulting in a reduction of fucosylated antigens on the cell surface. A recent study using fibroblasts from LADII/CDGIIc patients suggested that although terminal fucosylation of N-glycans is reduced severely, protein O-fucosylation is generally unaffected (Sturla, L., Rampal, R., Haltiwanger, R. S., Fruscione, F., Etzioni, A., and Tonetti, M. (2003) J. Biol. Chem. 278, 26727-26733). A potential explanation for this phenomenon is that enzymes adding O-fucose to proteins localize to cell organelles other than the Golgi apparatus. In this study, we investigated the subcellular localization of protein O-fucosyltransferase 1 (O-FucT-1), which is responsible for adding O-fucose to epidermal growth factor-like repeats. Our analysis reveals that, unlike all other known fucosyltransferases, O-FucT-1 is a soluble protein that localizes to the endoplasmic reticulum (ER). In addition, it appears that O-FucT-1 is retained in the ER by a KDEL-like sequence at its C terminus. Our results also suggest that enzymatic addition of O-fucose to proteins occurs in the ER, suggesting that a novel, ER-localized GDP-fucose transporter may exist. The fact that O-FucT-1 recognizes properly folded epidermal growth factor-like repeats, together with this unique localization, suggests that it may play a role in quality control."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m414574200"xsd:string
http://purl.uniprot.org/citations/15653671http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m414574200"xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/author"Luo Y."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/author"Luo Y."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/author"Haltiwanger R.S."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/author"Haltiwanger R.S."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/date"2005"xsd:gYear
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/date"2005"xsd:gYear
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/name"J. Biol. Chem."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/name"J. Biol. Chem."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/pages"11289-11294"xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/pages"11289-11294"xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/title"O-fucosylation of notch occurs in the endoplasmic reticulum."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/title"O-fucosylation of notch occurs in the endoplasmic reticulum."xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/volume"280"xsd:string
http://purl.uniprot.org/citations/15653671http://purl.uniprot.org/core/volume"280"xsd:string
http://purl.uniprot.org/citations/15653671http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/15653671
http://purl.uniprot.org/citations/15653671http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/15653671
http://purl.uniprot.org/citations/15653671http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/15653671
http://purl.uniprot.org/citations/15653671http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/15653671
http://purl.uniprot.org/uniprot/Q6EV70http://purl.uniprot.org/core/citationhttp://purl.uniprot.org/citations/15653671
http://purl.uniprot.org/uniprot/Q6EV70#attribution-CDF181F78C0B0C77D7A0ABA4CBC4C57Chttp://purl.uniprot.org/core/sourcehttp://purl.uniprot.org/citations/15653671