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Konrad Sandhoff

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Christina Ginkel; Dieter Hartmann; Mariona Rabionet; Konrad Sandhoff; Roger Sandhoff; Katharina vom Dorp; Joachim Degen; Ekrem Dere; Peter Dörmann; Matthias Eckhardt; Hany Farwanah; Armin Zlomuzica; Klaus Willecke
Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes.
Matthias Schäfer; Ann-Helen Willrodt; Wilhelm Bloch; Hany Farwanah; Daniel Hohl; Aaron J Huebner; Dennis Roop; Konrad Sandhoff; Sabine Werner
Nrf2 links epidermal barrier function with antioxidant defense.
Konrad Sandhoff
My journey into the world of sphingolipids and sphingolipidoses.

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All Publications

2012: Christina Ginkel; Dieter Hartmann; Mariona Rabionet; Konrad Sandhoff; Roger Sandhoff; Katharina vom Dorp; Joachim Degen; Ekrem Dere; Peter Dörmann; Matthias Eckhardt; Hany Farwanah; Armin Zlomuzica; Klaus Willecke
Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes.
The Journal of biological chemistry 2012;287(50):41888-902.
2012: Matthias Schäfer; Ann-Helen Willrodt; Wilhelm Bloch; Hany Farwanah; Daniel Hohl; Aaron J Huebner; Dennis Roop; Konrad Sandhoff; Sabine Werner
Nrf2 links epidermal barrier function with antioxidant defense.
EMBO molecular medicine 2012;4(5):364-79.
2012: Konrad Sandhoff
My journey into the world of sphingolipids and sphingolipidoses.
Proceedings of the Japan Academy. Series B, Physical and biological sciences 2012;88(10):554-82.
2011: Hany Farwanah; Thomas Kolter; Konrad Sandhoff
Mass spectrometric analysis of neutral sphingolipids: methods, applications, and limitations.
Biochimica et biophysica acta 2011;1811(11):854-60.
2011: Hichem D Gallala; Konrad Sandhoff
Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion.
Neurochemical research 2011;36(9):1594-600.
2011: Heike Schulze; Konrad Sandhoff
Lysosomal lipid storage diseases.
Cold Spring Harbor perspectives in biology 2011;3(6):.
2011: Hichem D Gallala; Bernadette Breiden; Konrad Sandhoff
Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids.
Journal of neurochemistry 2011;116(5):702-7.
2011: Irfan Y Tamboli; Heike Hampel; Nguyen T Tien; Karen Tolksdorf; Bernadette Breiden; Paul M Mathews; Paul Saftig; Konrad Sandhoff; Jochen Walter
Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2011;31(5):1837-49.
2011: Alexander Schneede; Marion Willenborg; Judith Blanz; Bernadette Breiden; Susanne Brodesser; Mykola Domanskyy; Jörg Heeren; Maarit Hölttä-Vuori; Elina Ikonen; Jobst Landgrebe; Paul Saftig; Konrad Sandhoff; Christine K Schmidt; Eeva-Liisa Eskelinen
Role for LAMP-2 in endosomal cholesterol transport.
Journal of cellular and molecular medicine 2011;15(2):280-95.
2010: Anna Wähe; Bahram Kasmapour; Christoph Schmaderer; David Liebl; Konrad Sandhoff; Anders Nykjaer; Gareth Griffiths; Maximiliano Gutierrez
Golgi-to-phagosome transport of acid sphingomyelinase and prosaposin is mediated by sortilin.
Journal of cell science 2010;123(Pt 14):2502-11.
2010: Misbaudeen Abdul-Hammed; Bernadette Breiden; Matthew A Adebayo; Jonathan O Babalola; Günter Schwarzmann; Konrad Sandhoff
Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion.
Journal of lipid research 2010;51(7):1747-60.
2010: Thomas Kolter; Konrad Sandhoff
Lysosomal degradation of membrane lipids.
FEBS letters 2010;584(9):1700-12.
2010: Thomas Kirkegaard; Anke G Roth; Nikolaj H T Petersen; Ajay K Mahalka; Ole Dines Olsen; Irina Moilanen; Alicja Zylicz; Jens Knudsen; Konrad Sandhoff; Christoph Arenz; Paavo K J Kinnunen; Jesper Nylandsted; Marja Jäättelä
Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology.
Nature 2010;463(7280):549-53.
2009: Reinhard Bauer; André Voelzmann; Bernadette Breiden; Ute Schepers; Hany Farwanah; Ines Hahn; Franka Eckardt; Konrad Sandhoff; Michael Hoch
Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila.
The EMBO journal 2009;28(23):3706-16.
2009: Silke Imgrund; Dieter Hartmann; Hany Farwanah; Matthias Eckhardt; Roger Sandhoff; Joachim Degen; Volkmar Gieselmann; Konrad Sandhoff; Klaus Willecke
Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas.
The Journal of biological chemistry 2009;284(48):33549-60.
2009: Christian Bach; Sabine Gilch; Romina Rost; Alex D Greenwood; Marion Horsch; Glaucia N M Hajj; Susanne Brodesser; Axel Facius; Sandra Schädler; Konrad Sandhoff; Johannes Beckers; Christine Leib-Mösch; Hermann M Schätzl; Ina Vorberg
Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells.
The Journal of biological chemistry 2009;284(45):31260-9.
2009: Michaela Wendeler; Konrad Sandhoff
Hexosaminidase assays.
Glycoconjugate journal 2009;26(8):945-52.
2009: Hany Farwanah; Jennifer Wirtz; Thomas Kolter; Klaus Raith; Reinhard H H Neubert; Konrad Sandhoff
Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric pressure chemical ionization mass spectrometry for separation, detection and mass spectrometric profiling of neutral sphingolipids and cholesterol.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences 2009;877(27):2976-82.
2009: Ulrich Matzner; Bernadette Breiden; Günter Schwarzmann; Afshin Yaghootfam; Arvan L Fluharty; Andrej Hasilik; Konrad Sandhoff; Volkmar Gieselmann
Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy.
The Journal of biological chemistry 2009;284(14):9372-81.
2009: Sabata Martino; Ilaria di Girolamo; Chiara Cavazzin; Roberto Tiribuzi; Rossella Galli; Anna Rivaroli; MANUELA VALSECCHI; Konrad Sandhoff; Sandro Sonnino; Angelo L Vescovi; Angela Gritti; Aldo Orlacchio
Neural precursor cell cultures from GM2 gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology.
Journal of neurochemistry 2009;109(1):135-47.
2009: Heike Schulze; Thomas Kolter; Konrad Sandhoff
Principles of lysosomal membrane degradation: Cellular topology and biochemistry of lysosomal lipid degradation.
Biochimica et biophysica acta 2009;1793(4):674-83.
2009: Christian Eggeling; Christian Ringemann; Rebecca Medda; Günter Schwarzmann; Konrad Sandhoff; Svetlana Polyakova; Vladimir Belov; Birka Hein; Claas von Middendorff; Andreas Schönle; Stefan W Hell
Direct observation of the nanoscale dynamics of membrane lipids in a living cell.
Nature 2009;457(7233):1159-62.
2008: Isabel Lastres-Becker; Susanne Brodesser; Dieter Lütjohann; Mekhman Azizov; Jana Buchmann; Edith Hintermann; Konrad Sandhoff; Annette Schürmann; Joachim Nowock; Georg Auburger
Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice.
Human molecular genetics 2008;17(10):1465-81.
2008: Maxim Rossmann; Robert Schultz-Heienbrok; Joachim Behlke; Natascha Remmel; Claudia Alings; Konrad Sandhoff; Wolfram Saenger; Timm Maier
Crystal structures of human saposins C andD: implications for lipid recognition and membrane interactions.
Structure (London, England : 1993) 2008;16(5):809-17.
2008: Roch-Philippe Charles; Marjorie Guitard; Céline Leyvraz; Bernadette Breiden; Marek Haftek; Zofia Haftek-Terreau; Jean-Christophe Stehle; Konrad Sandhoff; Edith Hummler
Postnatal requirement of the epithelial sodium channel for maintenance of epidermal barrier function.
The Journal of biological chemistry 2008;283(5):2622-30.
2007: Heike Schulze; Ute Schepers; Konrad Sandhoff
Overexpression and mass spectrometry analysis of mature human acid ceramidase.
Biological chemistry 2007;388(12):1333-43.
2007: Bernadette Breiden; Hichem Gallala; Thomas Doering; Konrad Sandhoff
Optimization of submerged keratinocyte cultures for the synthesis of barrier ceramides.
European journal of cell biology 2007;86(11-12):657-73.
2007: Natascha Remmel; Silvia Locatelli-Hoops; Bernadette Breiden; Guenter Schwarzmann; Konrad Sandhoff
Saposin B mobilizes lipids from cholesterol-poor and bis(monoacylglycero)phosphate-rich membranes at acidic pH. Unglycosylated patient variant saposin B lacks lipid-extraction capacity.
The FEBS journal 2007;274(13):3405-20.
2007: Jonathan O Babalola; Michaela Wendeler; Bernadette Breiden; Christoph Arenz; Guenter Schwarzmann; Silvia Locatelli-Hoops; Konrad Sandhoff
Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein.
Biological chemistry 2007;388(6):617-26.
2007: Hany Farwanah; Barbara Pierstorff; Christian E.H. Schmelzer; Klaus Raith; Reinhard H H Neubert; Thomas Kolter; Konrad Sandhoff
Separation and mass spectrometric characterization of covalently bound skin ceramides using LC/APCI-MS and Nano-ESI-MS/MS.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences 2007;852(1-2):562-70.
2007: Ralph Gareus; Marion Huth; Bernadette Breiden; Arianna Nenci; Nora Rösch; Ingo Haase; Wilhelm Bloch; Konrad Sandhoff; Manolis Pasparakis
Normal epidermal differentiation but impaired skin-barrier formation upon keratinocyte-restricted IKK1 ablation.
Nature cell biology 2007;9(4):461-9.
2007: Richard Jennemann; Roger Sandhoff; Lutz Langbein; Sylvia Kaden; Ulrike Rothermel; Hichem Gallala; Konrad Sandhoff; Herbert Wiegandt; Hermann-Josef Gröne
Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis.
The Journal of biological chemistry 2007;282(5):3083-94.
2007: Andreas Rummel; Timo Eichner; Tanja Weil; Tino Karnath; Aleksandrs Gutcaits; Stefan Mahrhold; Konrad Sandhoff; Richard L Proia; K Ravi Acharya; Hans Bigalke; Thomas Binz
Identification of the protein receptor binding site of botulinum neurotoxins B and G proves the double-receptor concept.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(1):359-64.
2006: Thomas Kolter; Konrad Sandhoff
Sphingolipid metabolism diseases.
Biochimica et biophysica acta 2006;1758(12):2057-79.
2006: Silvia Locatelli-Hoops; Natascha Remmel; Ralf Klingenstein; Bernadette Breiden; Maksim Rossocha; Maike Schoeniger; Christine Koenigs; Wolfram Saenger; Konrad Sandhoff
Saposin A mobilizes lipids from low cholesterol and high bis(monoacylglycerol)phosphate-containing membranes: patient variant Saposin A lacks lipid extraction capacity.
The Journal of biological chemistry 2006;281(43):32451-60.
2006: Michael John; Michaela Wendeler; Markus Heller; Konrad Sandhoff; Horst Kessler
Characterization of human saposins by NMR spectroscopy.
Biochemistry 2006;45(16):5206-16.
2006: Michaela Wendeler; Norbert Werth; Timm Maier; Guenter Schwarzmann; Thomas Kolter; Maike Schoeniger; Daniel Hoffmann; Thorsten Lemm; Wolfram Saenger; Konrad Sandhoff
The enzyme-binding region of human GM2-activator protein.
The FEBS journal 2006;273(5):982-91.
2006: Robert Schultz-Heienbrok; Natascha Remmel; Ralf Klingenstein; Maksim Rossocha; Konrad Sandhoff; Wolfram Saenger; Timm Maier
Crystallization and preliminary characterization of three different crystal forms of human saposin C heterologously expressed in Pichia pastoris.
Acta crystallographica. Section F, Structural biology and crystallization communications 2006;62(Pt 2):117-20.
2006: Florian Winau; Stephan Weber; subash sad; Juana de Diego; Silvia Locatelli Hoops; Bernadette Breiden; Konrad Sandhoff; Volker Brinkmann; Stefan H E Kaufmann; Ulrich E Schaible
Apoptotic vesicles crossprime CD8 T cells and protect against tuberculosis.
Immunity 2006;24(1):105-17.
2005: Thomas Kolter; Florian Winau; Ulrich E Schaible; Joachim Grötzinger; Konrad Sandhoff
Lipid-binding proteins in membrane digestion, antigen presentation, and antimicrobial defense.
The Journal of biological chemistry 2005;280(50):41125-8.
2005: Tadashi Yamashita; Maria Allende; Danielle N Kalkofen; Norbert Werth; Konrad Sandhoff; Richard L Proia
Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis.
Genesis (New York, N.Y. : 2000) 2005;43(4):175-80.
2005: Shahriar Koochekpour; Yu-Jun Zhuang; Rameen Beroukhim; Chia-Ling Hsieh; Matthias D Hofer; Haiyen E Zhau; Masao Hiraiwa; Daniel Y Pattan; Joy L Ware; Ronald B Luftig; Konrad Sandhoff; Charles L Sawyers; Kenneth J Pienta; Mark A Rubin; Robert L Vessella; William R Sellers; Oliver Sartor
Amplification and overexpression of prosaposin in prostate cancer.
Genes, chromosomes & cancer 2005;44(4):351-64.
2005: Guenter Schwarzmann; Michaela Wendeler; Konrad Sandhoff
Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system.
Glycobiology 2005;15(12):1302-11.
2005: Andrew J Norris; Julian P Whitelegge; Arman Yaghoubian; Jean-René Alattia; Gilbert G Privé; Tatsushi Toyokuni; Hubert Sun; Mai N Brooks; Luigi Panza; Pamela Matto; Federica Compostella; Natascha Remmel; Ralf Klingenstein; Konrad Sandhoff; Claire Fluharty; Arvan L Fluharty; Kym F Faull
A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A.
Journal of lipid research 2005;46(10):2254-64.
2005: Céline Leyvraz; Roch-Philippe Charles; Isabelle Rubera; Marjorie Guitard; Samuel Rotman; Bernadette Breiden; Konrad Sandhoff; Edith Hummler
The epidermal barrier function is dependent on the serine protease CAP1/Prss8.
The Journal of cell biology 2005;170(3):487-96.
2005: Irfan Y Tamboli; Kai Prager; Esther Barth; Michael T Heneka; Konrad Sandhoff; Jochen Walter
Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide.
The Journal of biological chemistry 2005;280(30):28110-7.
2005: Shahriar Koochekpour; Oliver Sartor; Masao Hiraiwa; Tae-Jin Lee; Walter Rayford; Natascha Remmel; Konrad Sandhoff; Ardalan Minokadeh; David Y Patten
Saposin C stimulates growth and invasion, activates p42/44 and SAPK/JNK signaling pathways of MAPK and upregulates uPA/uPAR expression in prostate cancer and stromal cells.
Asian journal of andrology 2005;7(2):147-58.
2005: Jose Antonio Chavez; William L Holland; Julia Bär; Konrad Sandhoff; Scott A Summers
Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling.
The Journal of biological chemistry 2005;280(20):20148-53.
2005: Tadashi Yamashita; Yun-Ping Wu; Roger Sandhoff; Norbert Werth; Hiroki Mizukami; Jessica M Ellis; Jeffrey L Dupree; Rudolf Geyer; Konrad Sandhoff; Richard L Proia
Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(8):2725-30.
2005: Thomas Kolter; Konrad Sandhoff
Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids.
Annual review of cell and developmental biology 2005;21():81-103.
2005: Peter Sawatzki; Ingo Damm; Barbara Pierstorff; Heike Hupfer; Konrad Sandhoff; Thomas Kolter
Site-specific cleavage--a model system for the identification of lipid-modified glutamate residues in proteins.
Chembiochem : a European journal of chemical biology 2005;6(1):178-85.
2004: Julia Reichelt; Bernadette Breiden; Konrad Sandhoff; Thomas M Magin
Loss of keratin 10 is accompanied by increased sebocyte proliferation and differentiation.
European journal of cell biology 2004;83(11-12):747-59.
2004: Hichem Gallala; Oliver Macheleidt; Thomas Doering; Volker Schreiner; Konrad Sandhoff
Nitric oxide regulates synthesis of gene products involved in keratinocyte differentiation and ceramide metabolism.
European journal of cell biology 2004;83(11-12):667-79.
2004: Melanie Kölzer; Klaus Ferlinz; Oliver Bartelsen; Silvia Locatelli Hoops; Florian Lang; Konrad Sandhoff
Functional characterization of the postulated intramolecular sphingolipid activator protein domain of human acid sphingomyelinase.
Biological chemistry 2004;385(12):1193-5.
2004: Madanan Madathiparambil Gopalakrishnan; Hans-Wilhelm Grosch; Silvia Locatelli-Hoops; Norbert Werth; Eva Smolenová; Michael Nettersheim; Konrad Sandhoff; Andrej Hasilik
Purified recombinant human prosaposin forms oligomers that bind procathepsin D and affect its autoactivation.
The Biochemical journal 2004;383(Pt. 3):507-15.
2004: Shahriar Koochekpour; Oliver Sartor; Tae-Jin Lee; Arthur W Zieske; David Y Patten; Masao Hiraiwa; Konrad Sandhoff; Natascha Remmel; Ardalan Minokadeh
Prosaptide TX14A stimulates growth, migration, and invasion and activates the Raf-MEK-ERK-RSK-Elk-1 signaling pathway in prostate cancer cells.
The Prostate 2004;61(2):114-23.
2004: Rosaria Buccoliero; Jacques Bodennec; Gerhild van Echten-Deckert; Konrad Sandhoff; Anthony H Futerman
Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease.
Journal of neurochemistry 2004;90(1):80-8.
2004: Brandy A Wicklow; Jennifer L Ivanovich; Margot M Plews; Timothy J Salo; Michael J Noetzel; Gregg T Lueder; Luca Cartegni; Michael M Kaback; Konrad Sandhoff; Robert D Steiner; Barbara L Triggs-Raine
Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNA.
American journal of medical genetics. Part A 2004;127A(2):158-66.
2004: Michaela Wendeler; Joerg Hoernschemeyer; Michael John; Norbert Werth; Maike Schoeniger; Thorsten Lemm; Rudolf Hartmann; Horst Kessler; Konrad Sandhoff
Expression of the GM2-activator protein in the methylotrophic yeast Pichia pastoris, purification, isotopic labeling, and biophysical characterization.
Protein expression and purification 2004;34(1):147-57.
2004: Melanie Kölzer; Norbert Werth; Konrad Sandhoff
Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine.
FEBS letters 2004;559(1-3):96-8.
2004: Michaela Wendeler; Joerg Hoernschemeyer; Daniel Hoffmann; Thomas Kolter; Guenter Schwarzmann; Konrad Sandhoff
Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation.
European journal of biochemistry / FEBS 2004;271(3):614-27.
2004: Florian Winau; Vera Schwierzeck; Robert Hurwitz; Natascha Remmel; Peter Allan Sieling; Robert L Modlin; Steven A Porcelli; Volker Brinkmann; Masahiko Sugita; Konrad Sandhoff; Stefan H E Kaufmann; Ulrich E Schaible
Saposin C is required for lipid presentation by human CD1b.
Nature immunology 2004;5(2):169-74.
2003: Melanie Kölzer; Christoph Arenz; Klaus Ferlinz; Norbert Werth; Heike Schulze; Ralf Klingenstein; Konrad Sandhoff
Phosphatidylinositol-3,5-Bisphosphate is a potent and selective inhibitor of acid sphingomyelinase.
Biological chemistry 2003;384(9):1293-8.
2003: Xingxuan He; Nozomu Okino; Rajwinder Dhami; Arie Dagan; Shimon Gatt; Heike Schulze; Konrad Sandhoff; Edward H Schuchman
Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase.
The Journal of biological chemistry 2003;278(35):32978-86.
2003: Nozomu Okino; Xingxuan He; Shimon Gatt; Konrad Sandhoff; Makoto Ito; Edward H Schuchman
The reverse activity of human acid ceramidase.
The Journal of biological chemistry 2003;278(32):29948-53.
2003: Dori Pelled; Christian Riebeling; Gerhild van Echten-Deckert; Konrad Sandhoff; Anthony H Futerman
Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease.
Neuropathology and applied neurobiology 2003;29(4):341-9.
2003: Konrad Sandhoff; Thomas Kolter
Biosynthesis and degradation of mammalian glycosphingolipids.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences 2003;358(1433):847-61.
2003: Timm Maier; Norbert Strater; Christina Schuette; Ralf Klingenstein; Konrad Sandhoff; Wolfram Saenger
The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease.
Journal of molecular biology 2003;328(3):669-81.
2003: Hatice Asuman Ozkara; Konrad Sandhoff
A new point mutation (G412 to A) at the last nucleotide of exon 3 of hexosaminidase alpha-subunit gene affects splicing.
Brain & development 2003;25(3):203-6.
2003: Hatice Asuman Ozkara; Konrad Sandhoff
Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease.
Brain & development 2003;25(3):191-4.
2003: Tadashi Yamashita; Akira Hashiramoto; Martin Haluzik; Hiroki Mizukami; Shoshannah Beck; Aaron Norton; Mari Kono; Shuichi Tsuji; Jose L Daniotti; Norbert Werth; Roger Sandhoff; Konrad Sandhoff; Richard L Proia
Enhanced insulin sensitivity in mice lacking ganglioside GM3.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(6):3445-9.
2003: Raymund Betz; Konrad Sandhoff; Klaus-Dieter Fischer; Gerhild van Echten-Deckert
Detection and identification of Vav1 protein in primary cultured murine cerebellar neurons and in neuroblastoma cells (SH-SY5Y and Neuro-2a).
Neuroscience letters 2003;339(1):37-40.
2003: Farsaneh Sadeghlar; Natascha Remmel; Bernadette Breiden; Ralf Klingenstein; Guenter Schwarzmann; Konrad Sandhoff
Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts.
Biochimie 2003;85(3-4):439-48.
2003: Stephanie Lansmann; Christina Schuette; Oliver Bartelsen; Joerg Hoernschemeyer; Thomas Linke; Judith Weisgerber; Konrad Sandhoff
Human acid sphingomyelinase.
European journal of biochemistry / FEBS 2003;270(6):1076-88.
2003: Michaela Wendeler; Thorsten Lemm; Judith Weisgerber; Joerg Hoernschemeyer; Oliver Bartelsen; Ute Schepers; Konrad Sandhoff
Expression of recombinant human GM2-activator protein in insect cells: purification and characterization by mass spectrometry.
Protein expression and purification 2003;27(2):259-66.
2003: M Diallo; Christoph Arenz; Katja Schmitz; Konrad Sandhoff; Ute Schepers
Long endogenous dsRNAs can induce complete gene silencing in mammalian cells and primary cultures.
Oligonucleotides 2003;13(5):381-92.
2003: Michaela Wendeler; Helmut Reilaender; Joerg Hoernschemeyer; Guenter Schwarzmann; Thomas Kolter; Konrad Sandhoff
Recombinant ganglioside GM2 synthase--expression in insect cells and enzyme assay.
Methods in enzymology 2003;363():476-89.
2003: Stefan T Hepbildikler; Michaela Wendeler; Roger Sandhoff; Konrad Sandhoff
Interaction of the GM2 activator protein with sulfated and sialylated glycosphingolipids.
Methods in enzymology 2003;363():207-22.
2003: Mustapha Diallo; Christoph Arenz; Katja Schmitz; Konrad Sandhoff; Ute Schepers
RNA interference: analyzing the function of glycoproteins and glycosylating proteins in mammalian cells.
Methods in enzymology 2003;363():173-90.
2002: Sven Nätzker; Thomas Heinemann; Santiago Figueroa-Perez; Barbara Schnieders; Richard R Schmidt; Konrad Sandhoff; Gerhild van Echten-Deckert
Cis-4-methylsphingosine phosphate induces apoptosis in neuroblastoma cells by opposite effects on p38 and ERK mitogen-activated protein kinases.
Biological chemistry 2002;383(12):1885-94.
2002: Thomas Doering; Helmut Brade; Konrad Sandhoff
Sphingolipid metabolism during epidermal barrier development in mice.
Journal of lipid research 2002;43(10):1727-33.
2002: Lutz Elsen; Raymund Betz; Guenter Schwarzmann; Konrad Sandhoff; Gerhild van Echten-Deckert
Identification of ceramide binding proteins in neuronal cells: a critical point of view.
Neurochemical research 2002;27(7-8):717-27.
2002: Mylvaganam Jeyakumar; David Smith; Elena Eliott-Smith; Mario Cortina Borja; Gabriele Reinkensmeier; Terry D Butters; Thorsten Lemm; Konrad Sandhoff; V Hugh Perry; Raymond A Dwek; Frances M Platt
An inducible mouse model of late onset Tay-Sachs disease.
Neurobiology of disease 2002;10(3):201-10.
2002: Thomas Kolter; Richard L Proia; Konrad Sandhoff
Combinatorial ganglioside biosynthesis.
The Journal of biological chemistry 2002;277(29):25859-62.
2002: Valérie Laurent-Matha; Annick Lucas; Silke Huttler; Konrad Sandhoff; Marcel Garcia; Henri Rochefort
Procathepsin D interacts with prosaposin in cancer cells but its internalization is not mediated by LDL receptor-related protein.
Experimental cell research 2002;277(2):210-9.
2002: Oliver Macheleidt; Hans Wilhelm Kaiser; Konrad Sandhoff
Deficiency of epidermal protein-bound omega-hydroxyceramides in atopic dermatitis.
The Journal of investigative dermatology 2002;119(1):166-73.
2002: Hiroki Mizukami; Yide Mi; Ryuichi Wada; Mari Kono; Tadashi Yamashita; Yujing Liu; Norbert Werth; Roger Sandhoff; Konrad Sandhoff; Richard L Proia
Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.
The Journal of clinical investigation 2002;109(9):1215-21.
2002: Chi-Ming Li; Jae-Ho Park; Calogera M Simonaro; Xingxuan He; Ronald E Gordon; Adriana Haimovitz-Friedman; Desiree Ehleiter; François Paris; Katia Manova-Todorova; Stefan Hepbildikler; Zvi Fuks; Konrad Sandhoff; Richard Kolesnick; Edward H Schuchman; Stefan Hepbiloikler
Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes.
Genomics 2002;79(2):218-24.
2002: Stefan T Hepbildikler; Roger Sandhoff; Melanie Kolzer; Richard L Proia; Konrad Sandhoff
Physiological substrates for human lysosomal beta -hexosaminidase S.
The Journal of biological chemistry 2002;277(4):2562-72.
2001: Klaus Ferlinz; G Kopal; Katussevani Bernardo; Thomas Linke; J Bar; B Breiden; U Neumann; Florian Lang; Edward H Schuchman; Konrad Sandhoff
Human acid ceramidase: processing, glycosylation, and lysosomal targeting.
The Journal of biological chemistry 2001;276(38):35352-60.
2001: Christina Schuette; Judith Weisgerber; Konrad Sandhoff
Complete analysis of the glycosylation and disulfide bond pattern of human beta-hexosaminidase B by MALDI-MS.
Glycobiology 2001;11(7):549-56.
2001: Norbert Werth; Christina Schuette; G Wilkening; Thorsten Lemm; Konrad Sandhoff
Degradation of membrane-bound ganglioside GM2 by beta -hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids.
The Journal of biological chemistry 2001;276(16):12685-90.
2001: Hiromichi Kawai; Maria Allende; Ryuichi Wada; Mari Kono; K Sango; Chuxia Deng; T Miyakawa; Jacqueline N Crawley; Norbert Werth; U Bierfreund; Konrad Sandhoff; Richard L Proia
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures.
The Journal of biological chemistry 2001;276(10):6885-8.
2001: J Bär; Thomas Linke; Klaus Ferlinz; U Neumann; Edward H Schuchman; Konrad Sandhoff
Molecular analysis of acid ceramidase deficiency in patients with Farber disease.
Human mutation 2001;17(3):199-209.
2001: Thomas Linke; G Wilkening; Farsaneh Sadeghlar; H Mozcall; Katussevani Bernardo; Edward H Schuchman; Konrad Sandhoff
Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins.
The Journal of biological chemistry 2001;276(8):5760-8.
2001: Thomas Linke; G Wilkening; Stephanie Lansmann; H Moczall; Oliver Bartelsen; Judith Weisgerber; Konrad Sandhoff
Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins.
Biological chemistry 2001;382(2):283-90.
2001: Konrad Sandhoff
The GM2-gangliosidoses and the elucidation of the beta-hexosaminidase system.
Advances in genetics 2001;44():67-91.
2000: G Wilkening; Thomas Linke; G Uhlhorn-Dierks; Konrad Sandhoff
Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP.
The Journal of biological chemistry 2000;275(46):35814-9.
2000: A Potratz; S Hüttler; U Bierfreund; Richard L Proia; Koichi Suzuki; Konrad Sandhoff
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency.
Biochimica et biophysica acta 2000;1502(3):391-7.
2000: A Strelow; Katussevani Bernardo; Sabine Adam-Klages; Thomas Linke; Konrad Sandhoff; Martin Krönke; Dieter Adam
Overexpression of acid ceramidase protects from tumor necrosis factor-induced cell death.
The Journal of experimental medicine 2000;192(5):601-12.
2000: Farsaneh Sadeghlar; Konrad Sandhoff; Gerhild van Echten-Deckert
Cell type specific localization of sphingomyelin biosynthesis.
FEBS letters 2000;478(1-2):9-12.
2000: Ute Schepers; Thorsten Lemm; Volker Herzog; Konrad Sandhoff
Characterization of regulatory elements in the 5'-flanking region of the GM2 activator gene.
Biological chemistry 2000;381(7):531-44.
2000: Ryoichi Kase; U Bierfreund; A Klein; Thomas Kolter; Kouichi Utsumi; K Itoha; Konrad Sandhoff; Hitoshi Sakuraba
Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease.
Biochimica et biophysica acta 2000;1501(2-3):227-35.
2000: Katussevani Bernardo; Oleg Krut; Katja Wiegmann; D Kreder; M Micheli; R Schäfer; A Sickman; W E Schmidt; Jens Schröder; Helmut E Meyer; Konrad Sandhoff; Martin Krönke
Purification and characterization of a magnesium-dependent neutral sphingomyelinase from bovine brain.
The Journal of biological chemistry 2000;275(11):7641-7.
2000: Elena Romiti; Valeria Vasta; Elisabetta Meacci; Marta Farnararo; Thomas Linke; Klaus Ferlinz; Konrad Sandhoff; Paola Bruni
Characterization of sphingomyelinase activity released by thrombin-stimulated platelets.
Molecular and cellular biochemistry 2000;205(1-2):75-81.
2000: D Wrobe; M Henseler; S Huettler; Samuel Ignacio Pascual-Pascual; A Chabas; Konrad Sandhoff
A non-glycosylated and functionally deficient mutant (N215H) of the sphingolipid activator protein B (SAP-B) in a novel case of metachromatic leukodystrophy (MLD).
Journal of inherited metabolic disease 2000;23(1):63-76.
2000: F Knoll; Thomas Kolter; Konrad Sandhoff
Sphingolipid photoaffinity labels.
Methods in enzymology 2000;311():568-600.
2000: U Bierfreund; Thomas Kolter; Konrad Sandhoff
Sphingolipid hydrolases and activator proteins.
Methods in enzymology 2000;311():255-76.
2000: Thomas Linke; Stephanie Lansmann; Konrad Sandhoff
Purification of acid ceramidase from human placenta.
Methods in enzymology 2000;311():201-7.
2000: Stephanie Lansmann; Oliver Bartelsen; Konrad Sandhoff
Purification and characterization of recombinant human acid sphingomyelinase expressed in insect Sf21 cells.
Methods in enzymology 2000;311():149-56.
2000: Gottfried Pohlentz; C Kaes; Konrad Sandhoff
In vitro assays for enzymes of ganglioside synthesis.
Methods in enzymology 2000;311():82-94.
1999: Chi-Ming Li; Jae-Ho Park; Xingxuan He; Brynn Levy; F Chen; K Arai; DA Adler; Christine M Disteche; J Koch; Konrad Sandhoff; Edward H Schuchman
The human acid ceramidase gene (ASAH): structure, chromosomal location, mutation analysis, and expression.
Genomics 1999;62(2):223-31.
1999: Klaus Ferlinz; Thomas Linke; Oliver Bartelsen; M Weiler; Konrad Sandhoff
Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins.
Chemistry and physics of lipids 1999;102(1-2):35-43.
1999: Julia Reichelt; Thomas Doering; E Schnetz; Manige Fartasch; Konrad Sandhoff; A M Magin
Normal ultrastructure, but altered stratum corneum lipid and protein composition in a mouse model for epidermolytic hyperkeratosis.
The Journal of investigative dermatology 1999;113(3):329-34.
1999: Tadashi Yamashita; Ryuichi Wada; T Sasaki; Chuxia Deng; U Bierfreund; Konrad Sandhoff; Richard L Proia
A vital role for glycosphingolipid synthesis during development and differentiation.
Proceedings of the National Academy of Sciences of the United States of America 1999;96(16):9142-7.
1999: Thomas Kolter; Thomas Doering; G Wilkening; Norbert Werth; Konrad Sandhoff
Recent advances in the biochemistry of glycosphingolipid metabolism.
Biochemical Society transactions 1999;27(4):409-15.
1999: Wiebke Möbius; Volker Herzog; Konrad Sandhoff; Guenter Schwarzmann
Gangliosides are transported from the plasma membrane to intralysosomal membranes as revealed by immuno-electron microscopy.
Bioscience reports 1999;19(4):307-16.
1999: Wiebke Möbius; Volker Herzog; Konrad Sandhoff; Guenter Schwarzmann
Intracellular distribution of a biotin-labeled ganglioside, GM1, by immunoelectron microscopy after endocytosis in fibroblasts.
The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 1999;47(8):1005-14.
1999: Christina Schuette; Thomas Doering; Thomas Kolter; Konrad Sandhoff
The glycosphingolipidoses-from disease to basic principles of metabolism.
Biological chemistry 1999;380(7-8):759-66.
1999: Thomas Heinemann; Grit-Carsta Bulwin; J Randall; Barbara Schnieders; Konrad Sandhoff; Hans-Dieter Volk; Edgar L Milford; SR Gullans; Nalân Utku
Genomic organization of the gene coding for TIRC7, a novel membrane protein essential for T cell activation.
Genomics 1999;57(3):398-406.
1999: A Giehl; Thorsten Lemm; Oliver Bartelsen; Konrad Sandhoff; Alfred Blume
Interaction of the GM2-activator protein with phospholipid-ganglioside bilayer membranes and with monolayers at the air-water interface.
European journal of biochemistry / FEBS 1999;261(3):650-8.
1999: Thomas Doering; W M Holleran; A Potratz; G Vielhaber; P M Elias; Koichi Suzuki; Konrad Sandhoff
Sphingolipid activator proteins are required for epidermal permeability barrier formation.
The Journal of biological chemistry 1999;274(16):11038-45.
1999: Nalân Utku; Grit-Carsta Bulwin; S Beinke; Thomas Heinemann; Francisca Beato; J Randall; Barbara Schnieders; Konrad Sandhoff; Hans-Dieter Volk; Edgar L Milford; SR Gullans
The human homolog of Drosophila cornichon protein is differentially expressed in alloactivated T-cells.
Biochimica et biophysica acta 1999;1449(3):203-10.
1999: Thomas Doering; Richard L Proia; Konrad Sandhoff
Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice.
FEBS letters 1999;447(2-3):167-70.
1999: Yujing Liu; Ryuichi Wada; Hiromichi Kawai; K Sango; Chuxia Deng; T Tai; MP McDonald; K Araujo; Jacqueline N Crawley; U Bierfreund; Konrad Sandhoff; Koichi Suzuki; Richard L Proia
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder.
The Journal of clinical investigation 1999;103(4):497-505.
1999: U Bierfreund; Thorsten Lemm; A Hoffmann; G Uhlhorn-Dierks; Robert A Childs; CT Yuen; Ten Feizi; Konrad Sandhoff
Recombinant GM2-activator protein stimulates in vivo degradation of GA2 in GM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay.
Neurochemical research 1999;24(2):295-300.
1999: Hitoshi Sakuraba; Kohji Itoh; Michie Shimmoto; Kouichi Utsumi; Ryoichi Kase; Y Hashimoto; T Ozawa; Y Ohwada; G Imataka; M Eguchi; T Furukawa; U Schepers; Konrad Sandhoff
GM2 gangliosidosis AB variant: clinical and biochemical studies of a Japanese patient.
Neurology 1999;52(2):372-7.
1998: Meryem Bektas; Y Dullin; Thomas Wieder; T Kolter; Konrad Sandhoff; Reinhard Brossmer; P Ihrig; Constantin E Orfanos; Christoph C Geilen
Induction of apoptosis by synthetic ceramide analogues in the human keratinocyte cell line HaCaT.
Experimental dermatology 1998;7(6):342-9.
1998: G Wilkening; Thomas Linke; Konrad Sandhoff
Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators.
The Journal of biological chemistry 1998;273(46):30271-8.
1998: Thomas Kolter; Konrad Sandhoff
Glycosphingolipid degradation and animal models of GM2-gangliosidoses.
Journal of inherited metabolic disease 1998;21(5):548-63.
1998: Oliver Bartelsen; Stephanie Lansmann; M Nettersheim; Thorsten Lemm; Klaus Ferlinz; Konrad Sandhoff
Expression of recombinant human acid sphingomyelinase in insect Sf21 cells: purification, processing and enzymatic characterization.
Journal of biotechnology 1998;63(1):29-40.
1998: Konrad Sandhoff; Thomas Kolter; Gerhild van Echten-Deckert
Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell.
Annals of the New York Academy of Sciences 1998;845():139-51.
1998: Chi-Ming Li; S B Hong; G Kopal; Xingxuan He; Thomas Linke; Wu-Shiun Hou; J Koch; Shimon Gatt; Konrad Sandhoff; Edward H Schuchman
Cloning and characterization of the full-length cDNA and genomic sequences encoding murine acid ceramidase.
Genomics 1998;50(2):267-74.
1998: F Norflus; Cynthia J Tifft; MP McDonald; G Goldstein; Jacqueline N Crawley; A Hoffmann; Konrad Sandhoff; Koichi Suzuki; Richard L Proia
Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.
The Journal of clinical investigation 1998;101(9):1881-8.
1998: Christina Schuette; Thorsten Lemm; G J Glombitza; Konrad Sandhoff
Complete localization of disulfide bonds in GM2 activator protein.
Protein science : a publication of the Protein Society 1998;7(4):1039-45.
1998: Yujing Liu; Koichi Suzuki; J D Reed; A Grinberg; H Westphal; A Hoffmann; T Döring; Konrad Sandhoff; Richard L Proia
Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(5):2503-8.
1998: Gerhild van Echten-Deckert; Athanassios Giannis; A Schwarz; Anthony H Futerman; Konrad Sandhoff
1-Methylthiodihydroceramide, a novel analog of dihydroceramide, stimulates sphinganine degradation resulting in decreased de novo sphingolipid biosynthesis.
The Journal of biological chemistry 1998;273(2):1184-91.
1998: Birgit Brenner; Klaus Ferlinz; Heike Grassmé; Michael Weller; U Koppenhoefer; Johannes Dichgans; Konrad Sandhoff; Florian Lang; Erich Gulbins
Fas/CD95/Apo-I activates the acidic sphingomyelinase via caspases.
Cell death and differentiation 1998;5(1):29-37.
1998: Konrad Sandhoff; Thomas Kolter
Processing of sphingolipid activator proteins and the topology of lysosomal digestion.
Acta biochimica Polonica 1998;45(2):373-84.
1998: Thomas Kolter; Konrad Sandhoff
Recent advances in the biochemistry of sphingolipidoses.
Brain pathology (Zurich, Switzerland) 1998;8(1):79-100.
1997: Gerhild van Echten-Deckert; A Klein; Thomas Linke; Thomas Heinemann; Judith Weisgerber; Konrad Sandhoff
Turnover of endogenous ceramide in cultured normal and Farber fibroblasts.
Journal of lipid research 1997;38(12):2569-79.
1997: Heike Grassmé; Erich Gulbins; Birgit Brenner; Klaus Ferlinz; Konrad Sandhoff; K Harzer; Florian Lang; Thomas F Meyer
Acidic sphingomyelinase mediates entry of N. gonorrhoeae into nonphagocytic cells.
Cell 1997;91(5):605-15.
1997: Konrad Sandhoff; Thomas Kolter
Biochemistry of glycosphingolipid degradation.
Clinica chimica acta; international journal of clinical chemistry 1997;266(1):51-61.
1997: C Michel; Gerhild van Echten-Deckert; J Rother; Konrad Sandhoff; E Wang; Alfred H Merrill
Characterization of ceramide synthesis. A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide.
The Journal of biological chemistry 1997;272(36):22432-7.
1997: Yujing Liu; A Hoffmann; A Grinberg; H Westphal; MP McDonald; K M Miller; Jacqueline N Crawley; Konrad Sandhoff; Koichi Suzuki; Richard L Proia
Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(15):8138-43.
1997: Thomas Wieder; Christoph C Geilen; T Kolter; F Sadeghlar; Konrad Sandhoff; Reinhard Brossmer; P Ihrig; D Perry; Constantin E Orfanos; Yusuf A Hannun
Bcl-2 antagonizes apoptotic cell death induced by two new ceramide analogues.
FEBS letters 1997;411(2-3):260-4.
1997: Gerhild van Echten-Deckert; A Zschoche; T Bär; Richard R Schmidt; A Raths; Thomas Heinemann; Konrad Sandhoff
cis-4-Methylsphingosine decreases sphingolipid biosynthesis by specifically interfering with serine palmitoyltransferase activity in primary cultured neurons.
The Journal of biological chemistry 1997;272(25):15825-33.
1997: B Albrecht; Gottfried Pohlentz; Konrad Sandhoff; Guenter Schwarzmann
Synthesis and mass spectrometric characterization of digoxigenin and biotin labeled ganglioside GM1 and their uptake by and metabolism in cultured cells.
Chemistry and physics of lipids 1997;86(1):37-50.
1997: M Pennybacker; Christina Schuette; B Liessem; Stefan T Hepbildikler; J A Kopetka; M R Ellis; Rachel Myerowitz; Konrad Sandhoff; Richard L Proia
Evidence for the involvement of Glu-355 in the catalytic action of human beta-hexosaminidase B.
The Journal of biological chemistry 1997;272(12):8002-6.
1997: G J Glombitza; E Becker; Hans Wilhelm Kaiser; Konrad Sandhoff
Biosynthesis, processing, and intracellular transport of GM2 activator protein in human epidermal keratinocytes. The lysosomal targeting of the GM2 activator is independent of a mannose-6-phosphate signal.
The Journal of biological chemistry 1997;272(8):5199-207.
1997: Klaus Ferlinz; R Hurwitz; H Moczall; Stephanie Lansmann; Edward H Schuchman; Konrad Sandhoff
Functional characterization of the N-glycosylation sites of human acid sphingomyelinase by site-directed mutagenesis.
European journal of biochemistry / FEBS 1997;243(1-2):511-7.
1996: J Koch; S Gärtner; Chi-Ming Li; L E Quintern; K Bernardo; O Levran; D Schnabel; Robert J Desnick; Edward H Schuchman; Konrad Sandhoff
Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease.
The Journal of biological chemistry 1996;271(51):33110-5.
1996: Stephanie Lansmann; Klaus Ferlinz; R Hurwitz; Oliver Bartelsen; G Glombitza; Konrad Sandhoff
Purification of acid sphingomyelinase from human placenta: characterization and N-terminal sequence.
FEBS letters 1996;399(3):227-31.
1996: G Vielhaber; R Hurwitz; Konrad Sandhoff
Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor.
The Journal of biological chemistry 1996;271(50):32438-46.
1996: U Schepers; G Glombitza; Thorsten Lemm; A Hoffmann; A Chabas; P Ozand; Konrad Sandhoff
Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant.
American journal of human genetics 1996;59(5):1048-56.
1996: K Sango; MP McDonald; Jacqueline N Crawley; M L Mack; Cynthia J Tifft; E Skop; C M Starr; A Hoffmann; Konrad Sandhoff; Kinuko Suzuki; Richard L Proia
Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis.
Nature genetics 1996;14(3):348-52.
1996: M Pennybacker; B Liessem; H Moczall; Cynthia J Tifft; Konrad Sandhoff; Richard L Proia
Identification of domains in human beta-hexosaminidase that determine substrate specificity.
The Journal of biological chemistry 1996;271(29):17377-82.
1996: N Fujita; Kunihiko Suzuki; marie T vanier; Brian Popko; Nobuyo Maeda; A Klein; M Henseler; Konrad Sandhoff; H Nakayasu; Kinuko Suzuki
Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.
Human molecular genetics 1996;5(6):711-25.
1996: M Henseler; A Klein; G J Glombitza; K Suziki; Konrad Sandhoff
Expression of the three alternative forms of the sphingolipid activator protein precursor in baby hamster kidney cells and functional assays in a cell culture system.
The Journal of biological chemistry 1996;271(14):8416-23.
1996: C Zacharias; Gerhild van Echten-Deckert; E Wang; Alfred H Merrill; Konrad Sandhoff
The effect of fumonisin B1 on developing chick embryos: correlation between de novo sphingolipid biosynthesis and gross morphological changes.
Glycoconjugate journal 1996;13(2):167-75.
1996: Konrad Sandhoff; Thomas Kolter
Topology of glycosphingolipid degradation.
Trends in cell biology 1996;6(3):98-103.
1996: M Henseler; A Klein; M Reber; marie T vanier; P Landrieu; Konrad Sandhoff
Analysis of a splice-site mutation in the sap-precursor gene of a patient with metachromatic leukodystrophy.
American journal of human genetics 1996;58(1):65-74.
1995: B Liessem; G J Glombitza; F Knoll; J Lehmann; J Kellermann; F Lottspeich; Konrad Sandhoff
Photoaffinity labeling of human lysosomal beta-hexosaminidase B. Identification of Glu-355 at the substrate binding site.
The Journal of biological chemistry 1995;270(40):23693-9.
1995: K Sango; S Yamanaka; A Hoffmann; Y Okuda; A Grinberg; Heiner Westphal; MP McDonald; Jacqueline N Crawley; Konrad Sandhoff; Kinuko Suzuki; Richard L Proia
Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism.
Nature genetics 1995;11(2):170-6.
1995: Konrad Sandhoff; Thomas Kolter
[Glycolipids of the cell surface--biochemistry of their decomposition].
Die Naturwissenschaften 1995;82(9):403-13.
1995: K Horinouchi; S Erlich; Daniel P Perl; Klaus Ferlinz; C L Bisgaier; Konrad Sandhoff; Robert J Desnick; C L Stewart; Edward H Schuchman
Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease.
Nature genetics 1995;10(3):288-93.
1995: Klaus Ferlinz; R Hurwitz; M Weiler; Kunihiko Suzuki; Konrad Sandhoff; marie T vanier
Molecular analysis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease.
American journal of human genetics 1995;56(6):1343-9.
1995: K Bernardo; R Hurwitz; T Zenk; Robert J Desnick; Klaus Ferlinz; Edward H Schuchman; Konrad Sandhoff
Purification, characterization, and biosynthesis of human acid ceramidase.
The Journal of biological chemistry 1995;270(19):11098-102.
1994: Klaus Ferlinz; R Hurwitz; G Vielhaber; Kunihiko Suzuki; Konrad Sandhoff
Occurrence of two molecular forms of human acid sphingomyelinase.
The Biochemical journal 1994;301 ( Pt 3)():855-62.
1994: R Hurwitz; Klaus Ferlinz; Konrad Sandhoff
The tricyclic antidepressant desipramine causes proteolytic degradation of lysosomal sphingomyelinase in human fibroblasts.
Biological chemistry Hoppe-Seyler 1994;375(7):447-50.
1994: Konrad Sandhoff; A Klein
Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.
FEBS letters 1994;346(1):103-7.
1994: A Klein; M Henseler; C Klein; Kunihiko Suzuki; Klaus Harzer; Konrad Sandhoff
Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo.
Biochemical and biophysical research communications 1994;200(3):1440-8.
1994: C Zacharias; Gerhild van Echten-Deckert; M Plewe; R R Schmidt; Konrad Sandhoff
A truncated epoxy-glucosylceramide uncouples glycosphingolipid biosynthesis by decreasing lactosylceramide synthase activity.
The Journal of biological chemistry 1994;269(18):13313-7.
1994: R Hurwitz; Klaus Ferlinz; G Vielhaber; H Moczall; Konrad Sandhoff
Processing of human acid sphingomyelinase in normal and I-cell fibroblasts.
The Journal of biological chemistry 1994;269(7):5440-5.
1994: Konrad Sandhoff; G van Echten
Ganglioside metabolism: enzymology, topology and regulation.
Progress in brain research 1994;101():17-29.
1993: B Liessem; Athanassios Giannis; Konrad Sandhoff; M Nieger
[Synthesis of 2-acetamido-1,4-imino-1,2,4-tridesoxy-D-galactitol and competitive inhibition of human lysosomal beta-hexosaminidase A].
Carbohydrate research 1993;250(1):19-30.
1993: Konrad Sandhoff
Bioactive glycosphingolipids with differentiation-inducing activity toward leukemia cells.
Japanese journal of cancer research : Gann 1993;84(11):inside front cover.
1993: M Schröder; D Schnabel; R Hurwitz; E Young; Kunihiko Suzuki; Konrad Sandhoff
Molecular genetics of GM2-gangliosidosis AB variant: a novel mutation and expression in BHK cells.
Human genetics 1993;92(5):437-40.
1993: marie T vanier; Klaus Ferlinz; R Rousson; S Duthel; Pierre Louisot; Konrad Sandhoff; Kinuko Suzuki
Deletion of arginine (608) in acid sphingomyelinase is the prevalent mutation among Niemann-Pick disease type B patients from northern Africa.
Human genetics 1993;92(4):325-30.
1993: H Klima; A Klein; G van Echten; Guenter Schwarzmann; Kunihiko Suzuki; Konrad Sandhoff
Over-expression of a functionally active human GM2-activator protein in Escherichia coli.
The Biochemical journal 1993;292 ( Pt 2)():571-6.
1993: G van Echten; Konrad Sandhoff
Ganglioside metabolism. Enzymology, Topology, and regulation.
The Journal of biological chemistry 1993;268(8):5341-4.
1993: Konrad Sandhoff; G van Echten
Ganglioside metabolism--topology and regulation.
Advances in lipid research 1993;26():119-42.
1992: J Rother; G van Echten; Guenter Schwarzmann; Konrad Sandhoff
Biosynthesis of sphingolipids: dihydroceramide and not sphinganine is desaturated by cultured cells.
Biochemical and biophysical research communications 1992;189(1):14-20.
1992: P Antes; Guenter Schwarzmann; Konrad Sandhoff
Distribution and metabolism of fluorescent sphingosines and corresponding ceramides bearing the diphenylhexatrienyl (DPH) fluorophore in cultured human fibroblasts.
European journal of cell biology 1992;59(1):27-36.
1992: P Antes; Guenter Schwarzmann; Konrad Sandhoff
Detection of protein mediated glycosphingolipid clustering by the use of resonance energy transfer between fluorescent labelled lipids. A method established by applying the system ganglioside GM1 and cholera toxin B subunit.
Chemistry and physics of lipids 1992;62(3):269-80.
1992: Konrad Sandhoff; G van Echten; M Schröder; D Schnabel; Kunihiko Suzuki
Metabolism of glycolipids: the role of glycolipid-binding proteins in the function and pathobiochemistry of lysosomes.
Biochemical Society transactions 1992;20(3):695-9.
1992: W Fürst; Konrad Sandhoff
Activator proteins and topology of lysosomal sphingolipid catabolism.
Biochimica et biophysica acta 1992;1126(1):1-16.
1992: E C Mandon; I Ehses; J Rother; G van Echten; Konrad Sandhoff
Subcellular localization and membrane topology of serine palmitoyltransferase, 3-dehydrosphinganine reductase, and sphinganine N-acyltransferase in mouse liver.
The Journal of biological chemistry 1992;267(16):11144-8.
1992: C S Kuhn; J Lehmann; Konrad Sandhoff
Efficient photoaffinity labeling of human beta-hexosaminidase A. Synthesis and application of 3-azi-1-[(2-acetamido-2-deoxy-1-beta- D-glucopyranosyl)thio]- and -galactopyranosyl)thio]butane.
Bioconjugate chemistry 1992;3(3):230-3.
1992: H Iber; C Zacharias; Konrad Sandhoff
The c-series gangliosides GT3, GT2 and GP1c are formed in rat liver Golgi by the same set of glycosyltransferases that catalyse the biosynthesis of asialo-, a- and b-series gangliosides.
Glycobiology 1992;2(2):137-42.
1992: H Iber; G van Echten; Konrad Sandhoff
Fractionation of primary cultured cerebellar neurons: distribution of sialyltransferases involved in ganglioside biosynthesis.
Journal of neurochemistry 1992;58(4):1533-7.
1992: P Leinekugel; S Michel; Ernst Conzelmann; Konrad Sandhoff
Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease.
Human genetics 1992;88(5):513-23.
1991: Klaus Ferlinz; R Hurwitz; Konrad Sandhoff
Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A.
Biochemical and biophysical research communications 1991;179(3):1187-91.
1991: M Schröder; D Schnabel; Kunihiko Suzuki; Konrad Sandhoff
A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB.
FEBS letters 1991;290(1-2):1-3.
1991: H Klima; A Tanaka; D Schnabel; T Nakano; M Schröder; Kunihiko Suzuki; Konrad Sandhoff
Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein.
FEBS letters 1991;289(2):260-4.
1991: A Vogel; Guenter Schwarzmann; Konrad Sandhoff
Glycosphingolipid specificity of the human sulfatide activator protein.
European journal of biochemistry / FEBS 1991;200(2):591-7.
1991: D Schnabel; M Schröder; Konrad Sandhoff
Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease.
FEBS letters 1991;284(1):57-9.
1991: E C Mandon; G van Echten; R Birk; R R Schmidt; Konrad Sandhoff
Sphingolipid biosynthesis in cultured neurons. Down-regulation of serine palmitoyltransferase by sphingoid bases.
European journal of biochemistry / FEBS 1991;198(3):667-74.
1991: Edward H Schuchman; M Suchi; Tsutomu Takahashi; Konrad Sandhoff; Robert J Desnick
Human acid sphingomyelinase. Isolation, nucleotide sequence and expression of the full-length and alternatively spliced cDNAs.
The Journal of biological chemistry 1991;266(13):8531-9.
1991: H Holtschmidt; Konrad Sandhoff; H Y Kwon; Klaus Harzer; T Nakano; Kunihiko Suzuki
Sulfatide activator protein. Alternative splicing that generates three mRNAs and a newly found mutation responsible for a clinical disease.
The Journal of biological chemistry 1991;266(12):7556-60.
1991: H Holtschmidt; Konrad Sandhoff; W Fürst; H Y Kwon; D Schnabel; Kunihiko Suzuki
The organization of the gene for the human cerebroside sulfate activator protein.
FEBS letters 1991;280(2):267-70.
1991: E M Meier; Guenter Schwarzmann; W Fürst; Konrad Sandhoff
The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A.
The Journal of biological chemistry 1991;266(3):1879-87.
1991: H Iber; G van Echten; Konrad Sandhoff
Substrate specificity of alpha 2----3-sialyltransferases in ganglioside biosynthesis of rat liver golgi.
European journal of biochemistry / FEBS 1991;195(1):115-20.
1991: Ernst Conzelmann; Konrad Sandhoff
Biochemical basis of late-onset neurolipidoses.
Developmental neuroscience 1991;13(4-5):197-204.
1990: Guenter Schwarzmann; Konrad Sandhoff
Metabolism and intracellular transport of glycosphingolipids.
Biochemistry 1990;29(49):10865-71.
1990: W Fürst; J Schubert; Werner Machleidt; H E Meyer; Konrad Sandhoff
The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein.
European journal of biochemistry / FEBS 1990;192(3):709-14.
1990: H Iber; G van Echten; R A Klein; Konrad Sandhoff
pH-dependent changes of ganglioside biosynthesis in neuronal cell culture.
European journal of cell biology 1990;52(2):236-40.
1990: E M Meier; D Schummer; Konrad Sandhoff
Evidence for the presence of water within the hydrophobic core of membranes.
Chemistry and physics of lipids 1990;55(2):103-13.
1990: G van Echten; R Birk; G Brenner-Weiss; R R Schmidt; Konrad Sandhoff
Modulation of sphingolipid biosynthesis in primary cultured neurons by long chain bases.
The Journal of biological chemistry 1990;265(16):9333-9.
1990: B Röder; J Dabrowski; U Dabrowski; H Egge; J Peter-Katalinić; Guenter Schwarzmann; Konrad Sandhoff
The determination of phytosphingosine-containing globotriaosylceramide from human kidney in the presence of lactosylceramide.
Chemistry and physics of lipids 1990;53(1):85-9.
1990: G van Echten; H Iber; H Stotz; A Takatsuki; Konrad Sandhoff
Uncoupling of ganglioside biosynthesis by Brefeldin A.
European journal of cell biology 1990;51(1):135-9.
1990: A Tanaka; K Ohno; Konrad Sandhoff; I Maire; E H Kolodny; A Brown; Kunihiko Suzuki
GM2-gangliosidosis B1 variant: analysis of beta-hexosaminidase alpha gene abnormalities in seven patients.
American journal of human genetics 1990;46(2):329-39.
1989: L E Quintern; Edward H Schuchman; O Levran; M Suchi; Klaus Ferlinz; H Reinke; Konrad Sandhoff; Robert J Desnick
Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts.
The EMBO journal 1989;8(9):2469-73.
1989: H Iber; Konrad Sandhoff
Identity of GD1C, GT1a and GQ1b synthase in Golgi vesicles from rat liver.
FEBS letters 1989;254(1-2):124-8.
1989: M Schröder; H Klima; T Nakano; H Kwon; L E Quintern; S Gärtner; Kunihiko Suzuki; Konrad Sandhoff
Isolation of a cDNA encoding the human GM2 activator protein.
FEBS letters 1989;251(1-2):197-200.
1989: L E Quintern; T S Zenk; Konrad Sandhoff
The urine from patients with peritonitis as a rich source for purifying human acid sphingomyelinase and other lysosomal enzymes.
Biochimica et biophysica acta 1989;1003(2):121-4.
1989: H Iber; R Kaufmann; Gottfried Pohlentz; Guenter Schwarzmann; Konrad Sandhoff
Identity of GA1, GM1a and GD1b synthase in Golgi vesicles from rat liver.
FEBS letters 1989;248(1-2):18-22.
1989: T Nakano; Konrad Sandhoff; J Stümper; H Christomanou; Kunihiko Suzuki
Structure of full-length cDNA coding for sulfatide activator, a Co-beta-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator.
Journal of biochemistry 1989;105(2):152-4.
1989: G van Echten; Konrad Sandhoff
Modulation of ganglioside biosynthesis in primary cultured neurons.
Journal of neurochemistry 1989;52(1):207-14.
1988: Gottfried Pohlentz; D Klein; Guenter Schwarzmann; D Schmitz; Konrad Sandhoff
Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver.
Proceedings of the National Academy of Sciences of the United States of America 1988;85(19):7044-8.
1988: C Hohenschutz; Waltraut Friedl; K H Schlör; A Waheed; Ernst Conzelmann; Konrad Sandhoff; Peter Propping
Probable metachromatic leukodystrophy/pseudodeficiency compound heterozygote at the arylsulfatase A locus with neurological and psychiatric symptomatology.
American journal of medical genetics 1988;31(1):169-75.
1988: W Fürst; Werner Machleidt; Konrad Sandhoff
The precursor of sulfatide activator protein is processed to three different proteins.
Biological chemistry Hoppe-Seyler 1988;369(5):317-28.
1988: Konrad Sandhoff; L Quintern
[Sphingolipid storage diseases of the central nervous system: bases of biochemical and clinical heterogeneity].
Die Naturwissenschaften 1988;75(3):123-31.
1988: Gottfried Pohlentz; D Klein; D Schmitz; Guenter Schwarzmann; J Peter-Katalinić; Konrad Sandhoff
Biosynthesis of gangliosides from asialogangliosides in rat liver Golgi vesicles.
Biological chemistry Hoppe-Seyler 1988;369(1):55-63.
1987: L E Quintern; G Weitz; H Nehrkorn; J M Tager; A W Schram; Konrad Sandhoff
Acid sphingomyelinase from human urine: purification and characterization.
Biochimica et biophysica acta 1987;922(3):323-36.
1987: A Vogel; W Fürst; M A Abo-Hashish; M Lee-Vaupel; Ernst Conzelmann; Konrad Sandhoff
Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide.
Archives of biochemistry and biophysics 1987;259(2):627-38.
1987: D Klein; Gottfried Pohlentz; U Hinrichs; Konrad Sandhoff
Metabolism of ganglioside-amides in cultured human fibroblasts.
Biological chemistry Hoppe-Seyler 1987;368(11):1495-503.
1987: D Klein; Gottfried Pohlentz; Guenter Schwarzmann; Konrad Sandhoff
Substrate specificity of GM2 and GD3 synthase of Golgi vesicles derived from rat liver.
European journal of biochemistry / FEBS 1987;167(3):417-24.
1987: H K Yusuf; Guenter Schwarzmann; Gottfried Pohlentz; Konrad Sandhoff
Oligosialogangliosides inhibit GM2- and GD3-synthesis in isolated Golgi vesicles from rat liver.
Biological chemistry Hoppe-Seyler 1987;368(5):455-62.
1987: Ernst Conzelmann; Konrad Sandhoff
Glycolipid and glycoprotein degradation.
Advances in enzymology and related areas of molecular biology 1987;60():89-216.
1986: Peter Propping; Waltraut Friedl; M Huschka; K H Schlör; F Reimer; M Lee-Vaupel; Ernst Conzelmann; Konrad Sandhoff
The influence of low arylsulfatase A activity on neuropsychiatric morbidity: a large-scale screening in patients.
Human genetics 1986;74(3):244-8.
1985: M Driessen; G Weitz; E M Brouwer-Kelder; Wilma E Donker-Koopman; J Bastiaannet; Konrad Sandhoff; J A Barranger; J M Tager; A W Schram
The effect of detergents on immunoprecipitability of lysosomal sphingomyelinase.
Biochimica et biophysica acta 1985;841(1):97-102.
1985: G Weitz; M Driessen; E M Brouwer-Kelder; Konrad Sandhoff; J A Barranger; J M Tager; A W Schram
Soluble sphingomyelinase from human urine as antigen for obtaining anti-sphingomyelinase antibodies.
Biochimica et biophysica acta 1985;838(1):92-7.
1984: A W Schram; M Dreissen; J Bastiaannet; Wilma E Donker-Koopman; E M Brouwer-Kelder; G Weitz; J A Barranger; Konrad Sandhoff; J M Tager
Immunological studies on lysosomal sphingomyelinase: identification of a 28 000-Da component deficient in urine from patients with Niemann-Pick disease types A and B.
Bioscience reports 1984;4(12):1051-7.
1984: H K Yusuf; Gottfried Pohlentz; Guenter Schwarzmann; Konrad Sandhoff
Ganglioside biosynthesis in rat liver golgi apparatus: stimulation by phosphatidylglycerol and inhibition by tunicamycin.
Advances in experimental medicine and biology 1984;174():227-39.
1984: H K Yusuf; Gottfried Pohlentz; Konrad Sandhoff
Ganglioside biosynthesis in Golgi apparatus: new perspectives on its mechanism.
Journal of neuroscience research 1984;12(2-3):161-78.
1983: H K Yusuf; Gottfried Pohlentz; Konrad Sandhoff
Tunicamycin inhibits ganglioside biosynthesis in rat liver Golgi apparatus by blocking sugar nucleotide transport across the membrane vesicles.
Proceedings of the National Academy of Sciences of the United States of America 1983;80(23):7075-9.
1983: H K Yusuf; Gottfried Pohlentz; Guenter Schwarzmann; Konrad Sandhoff
Ganglioside biosynthesis in Golgi apparatus of rat liver. Stimulation by phosphatidylglycerol and inhibition by tunicamycin.
European journal of biochemistry / FEBS 1983;134(1):47-54.