Search Results - "Berger, Eric G"

Identification and functional analysis of a defect in the human ALG9 gene: Definition of congenital disorder of glycosylation type IL by FRANK, Christian G, GRUBENMANN, Claudia E, EYAID, Wafaa, BERGER, Eric G, AEBI, Markus, HENNET, Thierry

“…Defects of lipid-linked oligosaccharide assembly lead to alterations of N-linked glycosylation known as "type I congenital disorders of glycosylation" (CDG)…”
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Golgi enzymes are enriched in perforated zones of golgi cisternae but are depleted in COPI vesicles by Kweon, Hee-Seok, Beznoussenko, Galina V, Micaroni, Massimo, Polishchuk, Roman S, Trucco, Alvar, Martella, Oliviano, Di Giandomenico, Daniele, Marra, Pierfrancesco, Fusella, Aurora, Di Pentima, Alessio, Berger, Eric G, Geerts, Willie J C, Koster, Abraham J, Burger, Koert N J, Luini, Alberto, Mironov, Alexander A

“…In the most widely accepted version of the cisternal maturation/progression model of intra-Golgi transport, the polarity of the Golgi complex is maintained by…”
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Genome-wide analysis of the unfolded protein response in fibroblasts from congenital disorders of glycosylation type-I patients by Lecca, M. Rita, Wagner, Ulrich, Patrignani, Andrea, Berger, Eric G, Hennet, Thierry

“…ABSTRACT Congenital disorders of glycosylation (CDG) are a family of diseases characterized by defects of N‐linked glycosylation. In CDG‐I, several genetic…”
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GTP-Bound Forms of rab6 Induce the Redistribution of Golgi Proteins into the Endoplasmic Reticulum by Martinez, Olivier, Antony, Claude, Pehau-Arnaudet, Gerard, Berger, Eric G., Salamero, Jean, Goud, Bruno

“…rab6 is a ubiquitous ras-like GTPase involved in intra-Golgi transport. We have studied at both morphological and biochemical levels the behavior of Golgi…”
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Transition of galactosyltransferase 1 from trans-Golgi cisterna to the trans-Golgi network is signal mediated by Schaub, Beat E, Berger, Bea, Berger, Eric G, Rohrer, Jack

“…The Golgi apparatus (GA) is the organelle where complex glycan formation takes place. In addition, it is a major sorting site for proteins destined for various…”
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Fibrotic response in fibroblasts from congenital disorders of glycosylation by Lecca, M. Rita, Maag, Charlotte, Berger, Eric G., Hennet, Thierry

“…Congenital disorders of glycosylation (CDG) are characterized by a generalized underglycosylation of proteins. CDG is associated with multiple symptoms such as…”
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Tn-syndrome by Berger, Eric G

“…The idiopathic Tn-syndrome, formerly called ‘permanent mixed-field polyagglutinability’, is a rare hematological disorder characterized by the expression of…”
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Small Cargo Proteins and Large Aggregates Can Traverse the Golgi by a Common Mechanism without Leaving the Lumen of Cisternae by Mironov, Alexander A., Beznoussenko, Galina V., Nicoziani, Paolo, Martella, Oliviano, Trucco, Alvar, Kweon, Hee-Seok, Di Giandomenico, Daniele, Polishchuk, Roman S., Fusella, Aurora, Lupetti, Pietro, Berger, Eric G., Willie J. C. Geerts, Koster, Abraham J., Koert N. J. Burger, Luini, Alberto

“…Procollagen (PC)-I aggregates transit through the Golgi complex without leaving the lumen of Golgi cisternae. Based on this evidence, we have proposed that…”
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Deficiency of the first mannosylation step in the N-glycosylation pathway causes congenital disorder of glycosylation type Ik by Grubenmann, Claudia E., Frank, Christian G., Hülsmeier, Andreas J., Schollen, Els, Matthijs, Gert, Mayatepek, Ertan, Berger, Eric G., Aebi, Markus, Hennet, Thierry

“…Defects of N-linked glycosylation represent diseases with multiple organ involvements that are classified as congenital disorders of glycosylation (CDG). In…”
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Localization of three human polypeptide GalNAc-transferases in HeLa cells suggests initiation of O-linked glycosylation throughout the Golgi apparatus by Röttger, S, White, J, Wandall, H H, Olivo, J C, Stark, A, Bennett, E P, Whitehouse, C, Berger, E G, Clausen, H, Nilsson, T

“…O-glycosylation of proteins is initiated by a family of UDP-N-acetylgalactosamine:polypeptide N-acetylgalactos-aminyltransferases (GalNAc-T). In this study, we…”
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Mapping the distribution of Golgi enzymes involved in the construction of complex oligosaccharides by Rabouille, C, Hui, N, Hunte, F, Kieckbusch, R, Berger, E G, Warren, G, Nilsson, T

“…The distribution of beta 1,2 N-acetylglucosaminyltransferase I (NAGT I), alpha 1,3-1,6 mannosidase II (Mann II), beta 1,4 galactosyltransferase (GalT), alpha…”
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ALG12 mannosyltransferase defect in congenital disorder of glycosylation type Ig by GRUBENMANN, Claudia E, FRANK, Christian G, KJAERGAARD, Susanne, BERGER, Eric G, AEBI, Markus, HENNET, Thierry

“…In the endoplasmic reticulum (ER) of eukaryotes, N-linked glycans are first assembled on the lipid carrier dolichyl pyrophosphate. The GlcNAc sub(2)Man…”
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Ectopic localizations of Golgi glycosyltransferases by Berger, Eric G

“…Glycosyltransferases involved in N- and O-glycan chain elongation and termination are localized in the Golgi apparatus. Early evidence in support of this rule…”
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A Mutation in the Human Ortholog of the Saccharomyces cerevisiae ALG6 Gene Causes Carbohydrate-Deficient Glycoprotein Syndrome Type-Ic by Imbach, Timo, Burda, Patricie, Kuhnert, Peter, Wevers, Ron A., Aebi, Markus, Berger, Eric G., Hennet, Thierry

“…Carbohydrate-deficient glycoprotein syndrome (CDGS) represents a class of genetic diseases characterized by abnormal N-linked glycosylation. CDGS patients show…”
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The β1,3-Galactosyltransferase β3GalT-V Is a Stage-specific Embryonic Antigen-3 (SSEA-3) Synthase by Zhou, Dapeng, Henion, Timothy R., Jungalwala, Firoze B., Berger, Eric G., Hennet, Thierry

“…We have previously reported the molecular cloning of β1,3-galactosyltransferase-V (β3GalT-V), which catalyzes the transfer of Gal to GlcNAc-based acceptors…”
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Localization of α1,3-fucosyltransferase VI in Weibel–Palade bodies of human endothelial cells by Schnyder-Candrian, Silvia, Borsig, Lubor, Moser, René, Berger, Eric G.

“…Surface glycosylation of endothelial cells is relevant to various processes including coagulation, inflammation, metastasis, and lymphocyte homing. One of the…”
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The C-Disaccharide α-C(1→3)-Mannopyranoside of N-Acetylgalactosamine Is an Inhibitor of Glycohydrolases and of Human α-1,3-Fucosyltransferase VI. Its Epimer α-(1→3)-Mannopyranoside of N-Acetyltalosamine Is Not by Pasquarello, Carla, Picasso, Sylviane, Demange, Raynald, Malissard, Martine, Berger, Eric G, Vogel, Pierre

“…The radical C-glycosidation of (−)-(1S,4R,5R,6R)-6-endo-chloro-3-methylidene-5-exo-(phenylseleno)-7-oxabicyclo[2.2.1]heptan-2-one ((−)-4) with…”
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A β -1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine Synthase Activity Is Structurally Related to β -1,3-galactosyltransferases by Zhou, Dapeng, Dinter, Andre, Gallego, Ricardo Gutierrez, Kamerling, Johannis P., Johannes F. G. Vliegenthart, Berger, Eric G., Hennet, Thierry

“…Human and mouse cDNAs encoding a new β -1,3-N-acetylglucosaminyltransferase (β 3GnT) have been isolated from fetal and newborn brain libraries. The human and…”
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MPDU1 mutations underlie a novel human congenital disorder of glycosylation, designated type If by Schenk, Barbara, Imbach, Timo, Frank, Christian G., Grubenmann, Claudia E., Raymond, Gerald V., Hurvitz, Haggit, Raas-Rotschild, Annick, Luder, Anthony S., Jaeken, Jaak, Berger, Eric G., Matthijs, Gert, Hennet, Thierry, Aebi, Markus

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Chemo-Enzymatic synthesis of the galili epitope Galα(1→3)Galβ(1→4)GlcNAc on a homogeneously soluble PEG polymer by a multi-Enzyme system by Brinkmann, Nils, Malissard, Martine, Ramuz, Maud, Römer, Ulrike, Schumacher, Thomas, Berger, Eric G, Elling, Lothar, Wandrey, Christian, Liese, Andreas

“…The α-Gal trisaccharide Galα(1→3)Galβ(1→4)GlcNAc 11 was synthesized on a homogeneously soluble polymeric support (polyethylene glycol, PEG) by use of a…”
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