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FORSCHUNGSBERICHT 1999-2001

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Institut für Physiologische Chemie

Allgemeine Angaben:
Nußallee 11, 53115 Bonn
Telefon: 0228 / 73-2411 /12
Fax: 0228 / 73-2416
eMail: sekretariat@institut.physiochem.uni-bonn.de

Hauptamtliche Professoren:
Prof. Dr. rer. nat. Ernst Bause
Prof. Dr. med. Volkmar Gieselmann
Prof. Dr. rer. nat. Manfred Kempfle

Wissenschaftliches Personal:
Universitätsstellen
Dr. rer. nat Wilhelm Breuer
Dr. rer. nat. Matthias Eckhardt
Dipl.-Chem. Claudia Foltys
Dr. rer. nat. Sebastian Franken
Dipl. Chem. Simone Glomsda
Dr. rer. nat. Heinz Rudolf Hartmann
Dr. rer. nat. Hem Chandra Jha
Dr. rer. nat. Christoph Kaes
Dr. med. Joachim Kappler
Dipl.-Chem. Saeed Khoursandi
Dr. phil. (Ph.D.) Roger A. Klein
Dr. rer. nat. Ulrich Matzner
AOR Prof. Dr. rer. nat. Uwe Murawski
PD Dr. rer. nat. Gottfried Pohlentz
Dr. rer. nat. Frank Schestag
Dr. rer. nat. Andreas Schwarz
Dr. Oec.troph. Susanne Starcke
Dr. rer. nat. Christof Völker
Dr. rer. nat. Afshin Yaghootfam

Drittmittelstellen
Dr. rer. nat. Raquel Aparicio
Dr. rer. nat. Julia Bär
Dr. rer. nat. Achim Bartoschek
Dipl.-Oec.troph. Susanne Bongart
Dipl.-Chem. Benno Coßmann
Dr. rer. nat. Birgit Hardt
Dipl. Biol. Michaela Hauptmann
Dr. rer. nat. Serafim Kiriakidis
Dipl.-Phys. Victor Pacheco-Torres
Dipl. Chem. Peter Poeppel
Dipl. Biol. Volker Roßlenbroich
Dr. rer. nat. Kornelia Treml
Dipl. Chem. Astrid van Noven

Forschungsschwerpunkte:
Struktur und Funktion von Glykosidasen des "frühen" Oligosaccharid-processings
(Bause, Aparicio, ,Treml,Völker, Treml, Völker)

Katalysemechnismus und Spezifität von Oligosaccharyltransferasen
(Bause, Breuer, Hardt, ,Bartoschek, Bartoschek)

Lipidmetabolismus des Myelins
(Eckhardt)

Pathogenese von Lipidosen
(Franken)

Neurotrophic actions of proteoglycans on neurons
(Kappler)

Untersuchungen an und mit 4,6,8(14)-Trien-3-on Steroiden
(Kempfle, Foltys)

Struktur und Konformationsanalyse von Glykokonjugaten.
(Klein, Hartmann)

Dünnschicht- und Gaschromatographie von Lipiden und Fettsäuren unterschiedlicher Organe der Wüstenrennmaus
(Murawski)

Charakterisierung von Glykosyltransferasen des Golgi mit Hilfe von Neoglykolipiden
(Pohlentz, Kaes)

Lysosomal storage disorders
(Schwarz)

Besondere Forschungsförderung:
Aktives Zentrum, Aufbau und Membrantopologie des Oligosaccharyltransferase-Komplexes aus Schweineleber
(Bause, SFB 284, Teilprojekt A2)

Enzymologie und Molekularbiologie von processing-Glykosidasen
(Bause, Völker, SFB 284, Teilprojekt B3)

Forschungsverbund: "Gentherapie lysosomaler Speichererkrankungen -Versuch zur Behandlung der metachromatischen Leukodystrophie als Modell einer Erkrankung mit primär neurologischer Symptomatik"
(Gieselmann, BMBF)

In vitro Untersuchungen und Zellkulturstudien an Glykosyltransferase
(Pohlentz, Schmitz, SFB 284,Teilprojekt A5)

Molekulare Mechanismen der Pathogenese der metachromatischen Leukodystrophie
(Gieselmann, SFB 400, Teilprojekt E2)

Struktur- und Konformationsanalyse der Glykokonjugate
(Klein, SFB 284,Teilprojekt A1)

Untersuchungen zur Biosynthese normaler und mutanter Arylsulfatase A.
(Gieselmann, SFB 284, Teilprojekt B10)

Veröffentlichungen:

Albach V, Klein R.A, Schmitz B
Do rodent and human brains have different N-glycosylation patterns?
Biol.Chem. 382(2), 187-194

Arteel G, Franken S, Kappler J, Sies H
Binding of selenoprotein P to heparin: Characterization with surface plasmon resonance.
Biol.Chem.381, 265-268

Bause E, Hardt B
Oligosaccharyltransferase complex: Ribophorin I, ribophorin II, OST48 and DAD1.
Handbook of Glycosyltransferases and related Genes 83, 577-586 ed.Taniguchi N, Springer (Tokyo)

Breuer W, Klein R.A, Hardt B, Bartoschek A, Bause E
Oligosaccharyltransferase is highly specific for the hydroxy amino acid in Asn-Xaa-Thr/Ser.
FEBS Lett. 501, 106-110

Coenen R, Gieselmann V, Lüllmann-Rauch R
Morphological alterations in the inner ear of the arylsulfatase A-deficient mouse.
Acta Neuropathol. (Berl) 101, 491-498

D'Hooge R, Coenen R, Gieselmann V, Lüllman-Rauch R, DeDeyn P.P
Decline in brain stem auditory evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase deficient mice.
Brain Res. 847, 352-356

D'Hooge R, Hartmann D, Manil J, Colin F, Gieselmann V, DeDeyn P.P
Neuromotor alterations and cerebellar deficits in aged arylsulfatase A dificient mice.
Neurosci. Lett. 273, 93-95

D'Hooge R, van Dam D, Franck F, Gieselmann V, DeDeyn P.P
Brain Res. 907, 35-43

De Praeter C, Gerwig G.J, Bause E, Nuytinck L.K, Vliegenthart J.F.G, Breuer W, Kamerling J.P, Espeel M.F, Martin J.J.R, DePaepe A.M, Chun Chan N.W, Dacremont G.A, van Coster R.N
A novel disorder in a newborn caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.
Am.J.Hum.Genet. 66, 1744-1756

Eckhardt M, Barth H, Blöcker D, Aktories K
Binding of Clostridium botulinum C2 toxin to asparagine-linked complex and hybrid carbohydrates.
J. Biol. Chem. 275, 2328-2334

Eckhardt M, Bukalo O, Chazal G, Wang L, Goridis C, Schachner M, Gerardy-Schahn R, Cremer H, Dityatey A
Mice deficient in the polysialyltransferase ST8SialIV/PST-1 allow discrimination of the roles of neural cell adhesion molecule protein and polysialic acid in neural development and synaptic plasticity.
J.Neurosci. 20, 5234-5244

Eckhardt M, Gotza B, Gerardy-Schahn R
Membrane topology of the mammalian CMP-sialic acid transporter.
J. Biol. Chem. 274, 8779-8787

Gutberlet T, Dietrich U, Bradaczek H, Pohlentz G, Leopold K, Fischer W
Cardiolipin, alpha-D-glucopyranosyl, and L-lysylcardiolipin from Gram-positive bacteria: FAB MS, monofilm, and X-ray powder diffraction studies.
Biochim. Biophys. Acta 1463, 307-322

Hardt B, Aparicio R, Bause E
The ologisaccharyltransferase complex from pig liver: cDNA cloning, expression and functional characterization.
Glycoconjugate J. 17, 767-779

Hardt B, Aparicio R, Breuer W, Bause E
Analysis of structural signals conferring localization of pig OST48 to the endoplasmatic reticulum.
Biol. Chem. 382, 1039-1047

Herrmann S, Schestag F, Polten A, Kafert S, Penzien J, Zlotogora J, Baumann N, Gieselmann V
Characterization of four arylsulfatase A missense mutations G86D, Y201C, D255H, and E312D causing metachromatic leukodystrophy.
Am.J.Med.Genet. 91, 68-73

Jonsdottir S.O, Welsh W.J, Rasmussen K, Klein R.A
The critical role of force-fields in property prediction.
New J.Chem.1999,153-163

Kappler J, Franken S, Junghans U, Linke T, Hoffmann R, Müller H.W, Koch K.W
Circular dichroism and glycosaminoglycan-binding properties of the C-terminal basic polypeptide sequence of netrin-1.
Biochem. Biophys. Res. Commun. 271, 287-291

Klein R.A, Pacheco V
Binary diol-water systems studied by [17]O nuclear magnetic resonance spectroscopy. Interaction of the effect of diol structure on the [17]O-water chemical shift. Formation of networks of water molecules stabilised by weak.
J.Phys.Chem.A 105, 9298-9304

Lühn K, Wild M.K, Eckhard M, Gerardy-Schahn R, Vestweber D
The defective gene in leukocyte adhesion deficiency II encodes a putative GDP-fucose transporter.
Nature Genet. 28, 69-72

Lüllmann-Rauch R, Matzner U, Franken S, Hartmann D, Gieselmann V
Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase.
Histochem. Cell Biol. 116, 161-169

Matzner U, Habetha M, Gieselmann V
Retrovirally expressed human arylsulfatase A corrects the metabolic defect of aralsulfatase A-deficient mouse cells.
Gene Ther. 7, 805-812

Matzner U, Harzer K, Learish R.D, Barranger J.A, Gieselmann V
Long-term expression and transfer of arylsulfatase A into brain of arylsulfatase A-deficient mice transplanted with bone marrow expressing the arylsulfatse A cDNA from retroviral vector.
Gene Ther. 14, 1250-1257

Matzner U, Schestag F, Hartmann D, Lüllmann-Rauch R, D'Hooge R, DeDeyn P.P
Bone marrow stem cell gene therapy of arylsulfatase A-deficient mice, using an arylsulfatase A mutant that is hypersecreted from retrovirally transduced donor-type cells.
Hum. Gene Ther. 12, 1021-1033

Obermeier S, Rudloff S, Pohlentz G, Lentze M.J, Kunz C
Secretion of [13]C labelled oligosaccharides into human milk and infant's urine after an oral [13]C galactose load.
Isot. Environ Health S. 35, 119-125

Pohlentz G, Drees B
Neoglycolipids derived from phosphatidylethanolamine serve as probes in cell culture studies on glycolipid metabolism.
Biol. Chem. 381, 29-34

Pohlentz G, Kaes C, Sandhoff K
In vitro assays for enzymes of ganglioside synthesis.
Method Enzymol. 311, 82-94

Schierau A, Dietz F, Lange H, Schestag F, Parastar A, Gieselmann V
Interaction of arylsulfatase A with UDP-N-acetylglucosamine: lysosomal enzyme N-acetylglucosamine-1-phosphotransferase.
J.Biol.Chem. 274, 3651-3658

Schluff P, Flott Rahmel B, Gieselmann V, Zimmer P, Das A, Ulrich K
Localization of receptors for endocytosis of lysosomal enzymes on different brain cells
J.Inher.Met.Dis. 21, 313-317

Schott I, Hartmann D, Gieselmann V, Lüllmann-Rauch R
Sulfatide storage in visceral organs of arylsulfatase A-deficient mice.
Virchows Arch. 439, 90-96

Treml K, Meimaroglou D, Hentges A, Bause E
The alpha-and ß-subunits are required for expression of catalytic activity in the hetero-dimetric glucosidase II complex from human liver.
Glycobiol. 10, 493-502

Vogel C, Pohlentz G
Synthesis of alpha-D-glucopyranosyl-(1-3)-alpha-D-mannopyranosyl-(1-7)-4-methylumbelliferone, a fluorogenic substrate for endo-alpha-1,2-mannosidase.
J. Carbohydr. Chem. 19, 1247-1258

Windfuhr M, Manegold A, Mühlenhoff M, Eckhardt M, Gerardy-Schahn R
Molecular defects that cause loss of polysialic acid in the complementation group
J. Biol. Chem. 275, 32861-32870

Xiangrong L, Johnk C, Hartmann D, Schestag F, Kromer W, Gieselmann V
Enzymatic properties, tissue-specific expression, and lysosomal location of two highly homologous rat SULT1C2 sulfotransferases.
Biochem.Biophys.Res.Commun. 272, 242-250


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