• Sandhoff K, Kolter T (1996) Topology of glycosphingolipid degradation. Trends Cell Biol, 6, 98-103. [pdf]

• Kolter T, Sandhoff K (1999) Sphingolipids -Their Metabolic Pathways and the Pathobiochemistry of Neurodegenerative Diseases. Angew Chem Int Ed, 38, 1532-68. [pdf]

• Kolter T, Proia RL, Sandhoff K (2002) Combinatorial ganglioside biosynthesis. J Biol Chem, 277, 25859-62. [pdf]

• Kolter T, Sandhoff K (2005) Principles of lysosomal membrane digestion-stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids. Annu Rev Cell Dev Biol, 21, 81-103. [pdf]

• Kolter T, Winau F, Schaible UE, Leippe M, Sandhoff K (2005) Lipid binding proteins inmembrane digestion, antigen presentation and antimicrobial defense. J Biol Chem, 280, 41125-28. [pdf]

• Sandhoff K (2012) My journey into the world of sphingolipids and sphingolipidoses. (2012) Proc Jpn Acad Ser B Phys Biol Sci, 88, 554-82. [pdf]

• Breiden B, Sandhoff K (2014) The role of sphingolipid metabolism in cutaneous permeability barrier formation. Biochim Biophys Acta, 1841, 441-52. [pdf]

• Schulze H, Sandhoff K (2014) Sphingolipids and lysosomal pathologies. Biochim Biophys Acta, 1841, 799-810. [pdf]

• Schwarzmann G, Arenz C, Sandhoff K (2014) Labeled chemical biology tools for investigating sphingolipid metabolism, trafficking and interaction with lipids and proteins. Biochim Biophys Acta, 1841, 1161-73. [pdf]

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• Sandhoff R, Sandhoff K (2023) Neuronal ganglioside and glycosphingolipid (GSL) metabolism and disease: Cascades of secondary metabolic errors can generate complex pathologies (in LSDs). Adv Neurobiol, 29, 333-90.

• Kasper BS, Thomas C, Albers A, Kasper EM, Sandhoff K (2023) From amaurotic idiocy to biochemically defined lipid storage diseases: the first identification of GM1-Gangliosidosis. Free Neuropathol, 4, 4-12.

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• Lopergolo D, Berti G, Mari F, Bertini E, Rufa A, Battisti C, Sicurelli F, Renieri A, Federico A, Sandhoff K, Malandrini A (2022) A case of spastic paraplegia type 11 mimicking a GM2-gangliosidosis. Neurol Sci, 43, 2849-52.

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• Breiden B, Sandhoff K (2021) Corrigendum to: Emerging mechanisms of drug-induced phopholipidosis. Biol Chem, 403, 251.

• Breiden B, Sandhoff K (2021) Acid sphingomyelinase, a lysosomal and secretory phospholipase C, is a key for cellular phospholipidosis catabolism. Int J Mol Sci, 22, 9001.

• Sandhoff K (2021) Secondary ganglioside GM2 accumulation in mucopolysaccharidosis. Mol Genet Metab Rep, 28, 100789.

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• Breiden B, Sandhoff K (2020) Mechanism of secondary ganglioside and lipid accumulation in lysosomal disease. Int J Mol Sci, 21, 2566.

• Beck M, Moser HW, Sandhoff K (2020) Acid ceramidase deficiency: Farber lipogranulomatosis, spinal muscular atrophy associated with progressive myoclonic epilepsy and peripheral osteolysis. In Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease, Sixth Edition, ed. JM Pascual and RN Rosenberg, 547-557.

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• Breiden B, Sandhoff K (2019) Lysosomal glycosphingolipid storage diseases. Annu Rev Biochem, 88, 461-85.

• Anheuser S, Breiden B, Sandhoff K (2019) Membrane lipids and their degradation compounds control GM2 catabolism at intralysosomal luminal vesicles. J Lipid Res, 60, 1099-111.

• Anheuser S, Breiden B, Sandhoff K (2019) Ganglioside GM2 catabolism is inhibited by storage compounds of mucopolysaccharidoses and by cationic amphiphilic drugs. Mol Genet Metab, 128, 75-83.

• Breiden B, Sandhoff K (2019) Emerging mechanisms of drug-induced phospholipidosis. Biol Chem, 401, 31-46.

• Sen N-E, Arsovic A, Meierhofer D, Brodesser S, Oberschmidt C, Canet-Pons J, Kaya Z-E, Halbach MV, Gispert S, Sandhoff K, Auburger G (2019) In spino-cerebellar tissue, Ataxin-2 expansion affects ceramide-sphingomyelin metabolism. Int J Mol Sci, 20, E5854.

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• Bülow MH, Wingen C, Senyilmaz D, Gosejacob D, Sociale M, Bauer R, Schulze H, Sandhoff K, Teleman AA, Hoch M, Sellin J (2018) Unbalanced lipolysis results in lipotoxicity and mitochondrial damage in peroxisome-deficient Pex19 mutants. Mol Biol Cell, 29, 396-407.

• Sociale M, Wulf A-L, Breiden B, Klee K, Thielisch M, Eckardt F, Sellin J, Bülow MH, Löbbert S, Weinstock N, Voelzmann A, Schultze J, Sandhoff K, Bauer R (2018) Ceramide synthase Schlank is a transcriptional regulator adapting gene expression to energy requirements. Cell Rep, 22, 1-12.

• Breiden B, Sandhoff K (2018) Ganglioside metabolism and its inherited diseases. Meth Mol Biol, 1804, 97-141.

• Sandhoff R, Schulze H, Sandhoff K (2018) Ganglioside metabolism in health and disease. Prog Mol Biol Transl Sci, 156, 1-62.

• Bickert A, Kern P, van Uelft M, Herresthal S, Ulas T, Gutbrod K, Breiden B, Degen J, Sandhoff K, Schultze JL, Dörmann P, Hartmann D, Bauer R, Willecke K (2018) Inactivation of Ceramide Synthase 2 catalytic activity in mice affects transcription of genes involved in lipid metabolism and cell division. Biochim Biophys Acta, 1863, 734-49.

• Sandhoff R, Sandhoff K (2018) Emerging concepts of ganglioside metabolism. FEBS Lett, 592, 3835-64.

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• Abdul-Hameed M, Breiden B, Schwarzmann G, Sandhoff K (2017) Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase GBA1. J Lipid Res, 58, 563-77.

• Schonauer S, Körschen HG, Penno A, Rennhack A, Breiden B, Sandhoff K, Gutbrod K, Dörmann P, Raju DN, Haberkant P, Gerl MJ, Brügger B, Zigdon H, Vardi A, Futerman AH, Thiele C, Wachten D (2017) Identification of a feedback loop involving β-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher disease. J Biol Chem, 292, 6177-89.

• Graf CGF, Schulz C, Schmälzlein M, Heinlein C, Mönnich M, Perkams L, Püttner M, Boos I, Hessefort M, Lombana Sanchez JN, Weyand M, Steegborn C, Breiden B, Ross K, Schwarzmann G, Sandhoff K, Unverzagt C (2017) Synthetic glycoforms reveal carbohydrate-dependent bioactivity of human saposin D. Angew Chem Int Ed Engl, 56, 5252-7.

• Sellin J, Schulze H, Paradis M, Gosejacob D, Papan C, Shevchenko A, Psathaki OE, Paululat A, Thielisch M, Sandhoff K, Hoch M (2017) Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases. Dis Model Mech, 10, 737-50.

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• Breiden B, Schwarzmann G, Sandhoff K (2016) Zelluläre Lipidhomöostase. GIT Laborfachzeitschrift, 4/2016, 53–55.

• Sandhoff K (2016) Membrane lipids and sphingolipid activator proteins regulate lysosomal sphingolipid catabolism. Biochimie, 130, 146-51.

• Schwarzmann G, Breiden B, Sandhoff K (2016) Lysosomal lipid binding and transfer proteins. GIT Laboratory Journal Europe, 7-8/2016, 26–27.

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• Sandhoff K, Harzer K (2013) Gangliosides and gangliosidoses: Principles of molecular and metabolic pathogenesis. J Neurosci, 33, 10195–208.

• Yildiz Y, Hoffmann P, vom Dahl S, Breiden B, Sandhoff R, Niederau C, Horwitz M, Karlsson S, Filacamo M, Elstein D, Beck M, Sandhoff K, Mengel E, Gonzalez MC, Nöthen MM, Sidransky E, Zimran A, Mattheisen M (2013) Functional and genetic characterization of the nonlysosomal glucosylceramidase 2 as a modifier for Gaucher disease. Orphanet J Rare Dis, 8, 151.

• Piyanova A, Albayram O, Rossi CA, Farwanah H, Michel K, Nicotera P, Sandhoff K, Bilkei-Gorzo A (2013) Loss of CB1 receptors leads to decreased cathepsin D levels and accelerated lipofuscin accumulation in the hippocampus. Mech Ageing Dev, 134, 391-9.

• Saftig P, Sandhoff K (2013) Killing from the inside. Nature, 502, 312-3.

• Sandhoff K (2013) Metabolic and cellular bases of sphingolipidoses. Biochem Soc Trans, 41, 1562-8.

• Tecedor L, Stein C, Schultz M, Farwanah H, Sandhoff K, Davidson B (2013) CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells. J Neurosci, 33, 18065-79.

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• Fehrenschild D, Galli U, Breiden B, Bloch W, Schettina P, Brodesser S, Michels C, Guenschmann C, Sandhoff K, Niessen CM, Niemann C. (2012) TCF/Lef1 mediated control of lipid metabolism regulates skin barrier function. J Invest Dermatol, 132, 337-45.

• Schäfer M, Farwanah H, Willrodt AH, Huebner AJ, Sandhoff K, Roop D, Hohl D, Bloch W, Werner S (2012) Nrf2 links epidermal barrier function with antioxidant defense. EMBO Mol Med, 4, 364-79.

• Ginkel C, Hartmann D, vom Dorp K, Zlomuzica A, Farwanah H, Eckhardt M, Sandhoff R, Degen J, Rabionet M, Dere E, Dörmann P, Sandhoff K, Willecke K (2012) Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin associated glycoprotein in oligodendrocytes. J Biol Chem, 287, 41888-902.

• Sandhoff K (2012) My journey into the world of sphingolipids and sphingolipidoses. (2012) Proc Jpn Acad Ser B Phys Biol Sci, 88, 554-82.

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• Frateschi S, Camerer E, Crisante G, Rieser S, Membrez M, Charles RP, Beermann F, Stehle JC, Breiden B, Sandhoff K, Rotman S, Haftek M, Wilson A, Ryser S, Steinhoff M, Coughlin SR, Hummler E (2011) PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nat Commun, 2, 161.

• Tamboli I, Hampel H, Tien N, Tolksdorf K, Breiden B, Mathews P, Saftig P, Sandhoff K, Walter J (2011) Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Aß generation. J Neurosci, 31, 1837-49.

• Gallala, HD, Breiden B, Sandhoff K (2011) Regulation of the NPC-2 protein-mediated cholesterol trafficking by membrane lipids J. Neurochem, 116, 702-7.

• Schulze H, Sandhoff K (2011) Lysosomal lipid storage diseases. Cold Spring Harb Perspect Biol, 3, 287-305.

• Schneede A, Schmidt CK, Hölttä-Vuori M, Heeren J, Willenborg M, Blanz J, Domanskyy M, Breiden B, Brodesser S, Landgrebe J, Sandhoff K, Ikonen E, Saftig P, Eeva-Liisa Eskelinen (2011) Role for LAMP-2 in endosomal cholesterol transport. J Cell Mol Med, 15, 280-95.

• Gallala HD, Sandhoff K (2011) Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion. Neurochem Res, 36, 1594-600.

• Farwanah H, Kolter T, Sandhoff, K (2011) Mass spectrometric analysis of neutralsphingolipids: Methods, applications, and limitations. Biochim. Biophys. Acta, 1811, 854-60.

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• Eggeling C, Ringemann C, Medda R, Schwarzmann G, Sandhoff K, Polyakova S, Belov VN, Hein B, von Middendorff C, Schönle A, Hell SW (2009) Direct observation of the nanoscale dynamics of membrane lipids in a living cell. Nature, 457, 1159-62.

• Martino S, Gritti A, di Girolamo I, Cavazzin C, Tiribuzi R, Galli R, Rivaroli A, Valsecchi M, Sandhoff K, Sonnino S, Vescovi A, and Orlacchio A (2009) Neural precursor cell cultures from GM2-gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology. J Neurochem, 109, 135-47.

• Matzner U, Breiden B, Schwarzmann G, Yaghootfam A, Fluharty AL, Hasilik A, Sandhoff K, Gieselmann V (2009) Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy. J Biol Chem, 284, 9372-81.

• Schulze H, Kolter T, Sandhoff K (2009) Principles of lysosomal membrane degradation. Biochim Biophys Acta, 1793, 674-83.

• Farwanah H, Wirtz J, Kolter T, Raith K, Neubert RHH, Sandhoff K (2009) Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric chemical ionization mass spectrometry for separation,detection and mass spectrometric profiling of neutral sphingolipids and cholesterol. J Chromatogr B Analyt Technol Biomed Life Sci, 877, 2976-82.

• Schulze H, Sandhoff K (2009) Farber’s Disease. In „Encyclopedia of Molecular Mechanisms of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 639.

• Kolter T, Schepers U, Sandhoff K (2009) Tay-Sachs‘ Disease in „Encyclopedic Reference of Molecular Mechanism of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 2027.

• Kolter T, Schepers U, Sandhoff K (2009) Sandhoff’s Disease in „Encyclopedic Reference of Molecular Mechanism of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 1881.

• Schepers U, Kolter T, Sandhoff K (2009) GM2-Activator Protein Deficiency in „Encyclopedia of Molecular Mechanisms of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 737.

• Schepers U, Kolter T, Sandhoff K (2009) SAP-precursor deficiency in „Encyclopedia of Molecular Mechanisms of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 1884.

• Schepers U, Kolter T, Sandhoff K (2009) Saposin B (SAP B) deficiency in „Encyclopedia of Molecular Mechanisms of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 1882.

• Schepers U, Kolter T, Sandhoff K (2009) Saposin C (SAP C) deficiency in „Encyclopedia of Molecular Mechanisms of Disease“, Vol 1, ed. Florian Lang, Springer-Verlag GmbH Berlin Heidelberg, p. 1883.

• Bach C, Gilch S, Rost R, Greenwood A, Horsch M, Hajj G, Brodesser S, Facius A, Schädler S, Sandhoff K, Beckers J, Leib-Moesch C, Schätzl HM, Vorberg I (2009) Prion-induced activation of cholesterogenic gene expression by a sterol regulatory binding protein (SREBP2) in neuronal cells. J Biol Chem, 284, 31260-9.

• Bauer, R, Voelzmann, A, Breiden, B, Schepers, U, Farwanah, H, Hahn, I, Eckhardt, F, Sandhoff K, Hoch M (2009) Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. EMBO J, 28, 3706-16.

• Imgrund S, Hartmann D, Farwanah H, Eckhardt M, Sandhoff R, Degen J, Gieselmann V, Sandhoff K, Willecke K (2009) Adult ceramide synthase 2 (CerS2) deficient mice exhibit myelin sheath defects, cerebellar degeneration and hepatocarcinomas. J Biol Chem, 284, 33549-60.

• Locatelli-Hoops S, Kolter T, Sandhoff K (2009) Sphingolipid-inherited diseases of the central nervous system In: Lajtha, A. (Ed.), Handbook of Neurochemistry and Molecular Neurobiology - Neural Lipids, 3rd Ed., Springer, 671-701.

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• Charles RP, Guitard M, Leyvraz C, Breiden B, Haftek M, Haftek-Terreau Z, Stehle JC, Sandhoff K, Hummler E (2008) Postnatal requirement of the epithelial sodium channel ENaC for the maintenance of the epidermal barrier function. J Biol Chem, 283, 2622-30.

• Lastres-Becker I, Brodesser S, Lütjohann D, Azizov M, Buchmann J, Hintermann E, Sandhoff K, Schuermann A, Nowock J, Auburger G (2008) Insulin receptor and lipid metabolism pathology in Ataxin 2 knock-out mice Hum. Mol Genetics, 17, 1465-81.

• Rossmann M, Schultz-Heienbrok R, Behlke J, Remmel N, Alings C, Sandhoff K, Saenger W, Maier T (2008) Crystal structures of human saposins C and D: implications for lipid recognition and membrane interactions. Structure 16, 809-17.

• Wendeler M, Sandhoff K (2008) Hexosaminidase assays. Glycoconj J, 26, 945-52.

• Sandhoff K (2008) From the big bang to the development of conciousness - self-organization of Matter. Prof. Kamaluddin Ahmad Memorial Lecture Proc. Golden Jub. Int. Sci. Conf. Vol1, pp1-20.

• Gallala, HD, Sandhoff K (2008) Principals of microdomain formation in biological membranes - Are there liquid ordered domains in living cellular membranes? Trends Glycosci Glycotechnol, 20, 277-95.

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• Winau F, Weber S, Sad S, de Diego J, Locatelli-Hoops S, Breiden B, Sandhoff K, Brinkmann V, Kaufmann SHE, Schaible UE (2006) Apopoptic vesicles crossprime CD8 T cells and protect against tuberculosis. Immunity, 24, 105-17.

• Wendeler M, Werth N, Maier T, Schwarzmann G, Kolter T, Schoeniger M, Hoffmann D, Lemm T, Saenger W, Sandhoff K (2006) The enzyme-binding region of human GM2-activator protein. FEBS J, 273, 982-91.

• Schultz-Heienbrok R, Remmel N, Klingenstein R, Rossocha M, Sandhoff K, Saenger W, Maier T (2006) Crystallization and preliminary characterization of three different crystal forms of human saposin C heterologously expressed in Pichia pastoris. Acta Crystallograph Sect F Struct Biol Cryst Commun, 62, 117-20.

• John M, Wendeler M, Heller M, Sandhoff K, Kessler H (2006) Characterization of human saposins by NMR spectroscopy. Biochemistry, 45, 5206-16.

• Locatelli-Hoops S, Kolter T, Sandhoff K (2006) Saposin C and other sphingolipid activator proteins. Gaucher Disease, eds. Anthony H. Futerman and Ari Zimran, CRC Press, Taylor & Francis Group, Chapter 4, 67-84

• Locatelli-Hoops S, Remmel N, Klingenstein R, Breiden B, Rossocha M, Schoeniger M, Koenigs C, Saenger W, Sandhoff K (2006) Saposin A mobilizes lipids from low cholesterol and high BMP containing membranes. patient variant saposin A lacks lipid extraction capacity. J Biol Chem, 281, 32451-60.

• Kolter T, Sandhoff K (2006) Sphingolipid metabolism diseases. Biochim Biophys Acta, 58, 2057-79.

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• Sawatzki P, Damm I, Pierstorff B, Hupfer H, Sandhoff K, Kolter T (2005) Site-specific cleavage - a model system for the identification of lipid-modified glutamate residues in proteins. Chembiochem, 6, 178-85

• Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM, Dupree JL, Geyer R, Sandhoff K, Proia R (2005) Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions. Proc Natl Acad Sci USA, 102, 2725-30.

• Chavez JA, Holland WL, Bär-Heine J, Sandhoff K, Summers SA (2005) Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling. J Biol Chem, 280, 20148-53

• Pavlu-Pereira H, Asfaw B, Poupctova H, Ledvinova J, Sikora J, Vanier MT, Sandhoff K, Zeman J, Novotna Z, Chudoba D, Elleder M (2005) Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study. J Inherit Metab Dis 28, 203-27.

• Kolter T, Sandhoff K (2005) Principles of lysosomal membrane digestion-stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids. Annu Rev Cell Dev Biol, 21, 81-103.

• Koochekpour S, Sartor O, Hiraiw, M, Lee T J, Rayford W, Remmel N, Sandhoff K, Minokadeh A, Patten DY (2005) 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 J Androl, 7, 147-58.

• Martino S, Marconi P, Tancini B, Dolcetta D, Cusella de Angelis MG, Montanucci P, Bregola G, Sandhoff K, Bordignon C, Manservigi R, Emiliani C, Orlacchio A (2005) A direct gene tranfer strategy via brain internal capsule reverses the biochemical defect in Tay-Sachs disease. Hum Mol Gen, 14, 2113-223.

• Tamboli IY, Prager K, Barth E, Heneka M, Sandhoff K, Walter J (2005) Inhibition of glucosphingolipid biosynthesis reduces secretion of the beta amyloid precursor protein and amyloid beta peptide. J Biol Chem, 280, 28110-17.

• Norris AJ, Whitelegge JP, Yaghoubian A, Alattia JR, Privé GG, Toyokuni T, Sun H, Brooks MN, Panza L, Matto P, Compostella F, Remmel N, Klingenstein R, Sandhoff K, Fluharty C, Fluharty A, Faull KW (2005) A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A. J Lipid Res, 46, 2254-64.

• Leyvraz C, Rubera I, Guitard M, Rotman S, Breiden B, Sandhoff K, Hummler E (2005) The epidermal barrier function is dependent on the serine protease CAP1/Prss8. J Cell Biol, 170, 487-96.

• Schwarzmann G, Wendeler M, Sandhoff K (2005) Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer-activity of GM2-activator protein by a FRET-based assay system. Glycobiology, 15, 1302-11.

• Koochekpour S, Zhuang YJ, Beroukhim R, Hsieh CL, Hofer MD, Zhau HE, Hiraiwa M, Pattan DY, Ware JL, Luftig RB, Sandhoff K, Sawyers CL, Pienta KJ, Rubin MA, Vessella RL, Sellers WR, Sartor O (2005) Amplification and overexpression of prosaposin in prostate cancer. Genes Chromosomes Cancer, 44, 351-64.

• Kolter T, Sandhoff K (2005) Lysosomal glycosphingolipid storage diseases. Neuroglycobiology , eds. Minoru Fukuda, Urs Rutisbauer and Ronald Schnaar, Oxford University Press, Chapter 8, 199-223.

• Yamashita T, Allende ML, Kalkofen DN, Werth N, Sandhoff K, Proia RL (2005) Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis, 43, 175-80.

• Kolter T, Winau F, Schaible UE, Leippe M, Sandhoff K (2005) Lipid binding proteins in membrane digestion, antigen presentation and antimicrobial defense. J Biol Chem, 280, 41125-28.

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• Winau F, Schwierzeck V, Hurwitz R, Remmel N, Sieling PA, Brinkmann V, Sandhoff K, Kaufmann SHE, Schaible E (2004) Saposin C is required for lipid presentation by human CD1b. Nat Immunol, 5, 169-74.

• Wendeler M, Hoernschemeyer J, Hoffmann D, Kolter T, Schwarzmann G, Sandhoff K (2004) Photoaffinity labelling of human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation. Eur J Biochem, 271, 614-27.

• Wendeler M, Hoernschemeyer J, John M, Werth N, Schoeniger M, Lemm T, Hartmann R, Kessler H, Sandhoff K (2004) Expression of the GM2-Activator Protein in the methylotrophic yeast pichia pastoris purification, isotopic labeling and biophysical characterization. Prot Exp Purif, 34,147-57.

• Kölzer M, Werth N, Sandhoff K (2004) Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine. FEBS Lett, 559, 96-8.

• Macheleidt O, Kolter T, Sandhoff K (2004) Defects in activator proteins and other soluble proteins of the lysosome in: Lysosomal Disorders of the Brain, eds. F.M. Platt and S.U.Walkley, Oxford University Press, Chapter 8, 186-99.

• Buccoliero R, Bodennec J, van Echten-Deckert G, Sandhoff K, Futerman AH (2004) Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease. J Neurochem, 90, 80-8.

• Koochekpour S, Sartor O, Lee TJ, Zieske A, Patten DY, Hiraiwa M, Sandhoff K, Remmel N, Minokadeh A (2004) Prosaptide TX4A stimulates growth, migration, and invasion and activates the Raf-MEK-ERK-RSK-ELK-I signaling pathway in prostate cancer cells. Prostate, 61, 114-23.

• Wicklow BA, Ivanovich J, Plews M, Salo T, Noetzel M, Lueder TG, Cartegni L, Kaback M, Sandhoff K, Steiner RD, Triggs-Raine BL (2004) Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNA. Am J Med Gen A, 127A, 158-66.

• Sawatzki P, Mikeska T, Sandhoff K, Nieger M, Kolter T (2004) 6(S)-Methyl-3(S)-(1-methylethyl)piperazin-2-one. Acta Cryst, E59, 171-73.

• Gopalakrishnan MM, Grosch HW, Locatelli-Hoops S, Werth N, Smolenova E, Nettershein M, Sandhoff K, Hasilik A (2004) Purified recombinant human prosaposin forms oliogomers that bind procathepsin D and affect its autoactivation. Biochem J 383, 507-15.

• Schueler UH, Kolter T, Kaneski CR, Zirzow GC, Sandhoff K, Brady RO (2004) Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. J Inherit Metab Dis 27, 649-58.

• Kölzer M, Ferlinz K, Bartelsen O, Locatelli-Hoops S, Sandhoff K (2004) Functional characterization of the postulated intramolcular sphingolipid activator protein domain of the human acid sphingomyelinase. Biol Chem, 385, 1193-5.

• Gallala H, Macheleidt O, Doering T, Schreiner V, Sandhoff K (2004) Nitric oxide modulates expression of the gene products involved in keratinocyte differentiation and ceramide metabolism. Eur J Cell Biol, 83, 667-79.

• Reichelt J, Breiden B, Sandhoff K, Magin T (2004) Loss of keratin 10 is accompanied by increased sebocyte proliferation and differentiation. Eur J Cell Biol, 83, 747-59.

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• Wendeler M, Lemm T, Weisgerber J, Hoernschemeyer J, Bartlesen O, Schepers U, Sandhoff K (2003) Expression of recombinant human GM2-activator protein in insect cells, purification and characterization by mass spectrometry. Protein Expr Purif, 27, 259-66.

• Lansmann S, Schuette CG, Bartelsen O, Hoernschemeyer J, Linke T, Weisgerber J, Sandhoff K (2003) Human acid sphingomyelinase - Assignment of the disulfide bond pattern. Eur J Biochem, 270, 1076-88.

• Betz R, Sandhoff K, Fischer KD, van Echten-Deckert G (2003) Detection and identification of Vav1 protein in primary cultured murine cerebellar neurons and in neuroblastoma cells. Neurosci Lett, 339, 37-40.

• Maier T, Strater N, Schuette C, Klingenstein R, Sandhoff K, Saenger W (2003) The X-Ray crystal structure of human Hexosaminidase B provides new insights into Sandhoff disease. J Mol Biol, 328, 669-81.

• Ozkara HA, Sandhoff K (2003) Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease. Brain Dev, 25, 191-94.

• Ozkara HA, Sandhoff K (2003) A new mutation (G412 to A) at the last nucleotide of exon 3 of hexosaminidase alpha-subunit gene affects splicing. Brain Dev, 25, 203-6.

• Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, Norton A, Kono M, Tsuji S, Daniotti JL, Werth N, Sandhoff R, Sandhoff K, Proia RL (2003) Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc Natl Acad Sci USA, 100, 3445-9.

• Sadeghlar F, Remmel N, Breiden B, Klingenstein R, Schwarzmann G, Sandhoff K (2003) Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts. Biochimie, 85, 439-48.

• Okino N, He X, Gatt S, Sandhoff K, Ito M, Schuchman EH (2003) The reverse activity of acid ceramidase. J Biol Chem, 278, 29948-53.

• Sandhoff K, Kolter T (2003) Biosynthesis and degradation of mammalian glycosphingolipids. Philos Trans R Soc Lond B Biol Sci, 358, 847-61.

• He X, Okino N, Dhami R, Dagan A, Gatt A, Gatt S, Schulze H, Sandhoff K, Schuchman EH (2003) Purification and characterization of recombinant, human acid ceramidase: Catalytic reactions and interactions with acid sphingomyelinase. J Biol Chem, 278, 32978-86.

• Pelled D, Riebeling C, van Echten-Deckert G, Sandhoff K, Futerman HA (2003) Reduced rates of axonal and dendritic growth in embryonic hipposcampal neurons cultured from a mouse model of Sandhoff disease. Neuropathol Appl Neurobiol, 29, 341-9.

• Mantipragada SB, Horváth LI, Arias HR, Schwarzmann G, Sandhoff K, Barrantes FJ, Marsh D (2003) Lipid-protein interactions and the effect of local anaesthetics in acetylcholine receptor-rich membranes from Torpedo marmorata electric organ. Biochemistry 42, 9167-75.

• Diallo M, Arenz C, Sandhoff K, Schepers U (2003) RNA Interference: A new method to analyse the function of glycoproteins and glycosylating proteins in mammalian cells. Knockout experiments with UDP-glucose/ceramid-glucosyltransferase. Methods Enzymol, 363, 173-90.

• Kölzer M, Arenz C, Ferlinz K, Werth N, Schulze H, Klingenstein R, Sandhoff K (2003) Phosphatidylinositol-3,5-bisphosphate is a potent andselective inhibitor of acid sphingomyelinase. Biol Chem, 384, 1293-8.

• Wendeler M, Reilaender H, Hoernschemeyer J, Schwarzmann G, Kolter T, Sandhoff K (2003) Recombinant ganglioside GM2-synthase - expression in insect cells and enzyme assay. Methods Enzymol, 363, 476-89.

• Hepbildikler ST, Wendeler M, Sandhoff R, Sandhoff K (2003) Interaction of GM2 activator protein with sulphated and sialylated glycosphingolpids. Methods Enzymol, 363, 207-22.

• Schueler UH, Kolter T, Kaneski CR, Blusztajn JK, Herkenham M, Sandhoff K, Brady RO (2003) Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3. Neurobiol Dis, 14, 595-601.

• Diallo M,Arenz C, Schmitz K, Sandhoff K, Schepers U (2003) Long endogenous dsRNAs can induce complete gene silencing in mammalian cells and primary cultures. Oligonucleotides, 13, 381-92.

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• Hepbildikler S, Sandhoff R, Kölzer, M, Proia RL, Sandhoff K (2002) Physiological substrates for human lysosomal -hexosaminidase S. J Biol Chem, 277, 2562-72.

• Li CM, Park JH, Simonaro CM, He XX, Gordon RE, Friedman AH, Ehleiter D, Paris F, Manova K, Hepbildikler S, Fuks Z, Sandhoff K, Kolesnick R, Schuchman EG (2002) Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics, 79, 218-24.

• Hüttler S, Sandhoff K (2002) Prosaposin. Wiley Encyclopedia of Molecular Medicine, Wiley & Sons, Vol.1, 724-7.

• Bär J, Sandhoff K (2002) Ceramidase. Wiley Encyclopedia of Molecular Medicine, Wiley & Sons, Vol.4, 2606-9.

• Schueler U, Kaneski C, Murray G, Sandhoff K, Brady CO (2002) Uptake of mannose-terminal glucocerebrosidase in cultured human cholinergic and dopaminergic neuron cell lines. Neurochem Res, 27, 325-30.

• Nettersheim M, Sandhoff K (2002) Sphingolipid activator proteins. Wiley Encyclopedia of Molecular Medicine, Wiley & Sons, Vol.5, 2973-5.

• Mizukami H, Mi Y, Wada R, Kono M, Yamashita T, Liu Y, Werth N, Sandhoff R, Sandhoff K, Proia RL (2002) Systemic inflammation in glucocerebrosidase deficient mice with minimal glucosylceramide storage. J Clin Invest, 109, 1215-21.

• Kolter T, Proia RL, Sandhoff K (2002) Combinatorial ganglioside biosynthesis. J Biol Chem, 277, 25859-62.

• Laurent-Matha V, Lucas A, Hüttler S, Sandhoff K, Garcia M, Rochefort H (2002) Procathepsin D interacts with prosaposin in cancer cells but its internalization is not mediated by LRP (LDL receptor related protein). Exp. Cell Res, 277, 210-9.

• Sandhoff K (2002) Glykosphingolipide als Membranbausteine - molekulare Ursachen neurodegenerativer und dermaler Erkrankungen. Jahrbuch 2001 der Deutschen Akademie der Naturforscher Leopoldina, LEOPOLDINA (R.3) 47, 415-35.

• Macheleidt O, Kaiser HW, Sandhoff K (2002) Deficiency of epidermal protein-bound w-hydroxyceramides in atopic dermatitis. Deficiency of epidermal protein-bound w-hydroxyceramides in atopic dermatitis. J Invest Dermatol, 119, 166-73.

• Jeyakumar M, Smith D, Eliott-Smith E, Cortina-Borja M, Reinkensmeier G, Butters TD, Lemm T, Sandhoff K, Perry VH, Dwek RA, Platt F (2002) An inducible mouse model of late onset Tay-Sachs disease. Neurobiol Dis, 10, 201-10.

• Martino S, Cavalieri C, Emiliani C, Dolcetta D, Cusella De Angelis MG, Chigorno V, Severini GM, Sandhoff K, Bordignon C, Sonnino S, Orlacchio A (2002) Restoration of the GM2 ganglioside metabolism in bone marrow-derived stromal cells from Tay-Sachs disease animal model. Neurochem Res, 27, 793-800.

• Elsen L, Betz R, Schwarzmann G, Sandhoff K, van Echten-Deckert G (2002) Identification of ceramide binding proteins in neuronal cells: A critical point of view. Neurochem Res, 27, 717-27.

• Doering T, Brade H, Sandhoff K (2002) Sphingolipid metabolism during epidermal barrier development in mice. J Lipid Res, 10, 1727-33.

• Nätzker S, Heinemann T, Figueroa-Perez S, Schnieders B, Schmidt RR, Sandhoff K, van Echten-Deckert G (2002) cis-4-Methylsphingosine-phosphate induces apoptosis in neuroblastoma cells by opposite effects on p38 and ERK mitogen-activated protein kinases. Biol Chem, 383, 1885-94.

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• Gravel RA, Clarke JTR, Kaback MM, Mahuran D, Sandhoff K, Suzuki K (2001) The GM2-gangliosidosis in: The Metabolic and Molecular Bases of Inherited Disease. Editions Scriver C, Beaudet A, Sly L, William. S, Valle D, 8th Edition, David Mc Graw Hill, N.Y., 8th Edition, Chapter 153, 3827-77.

• Sandhoff K, Kolter T, K Harzer (2001) Sphingolipid activator proteins, in: The Metabolic and Molecular Bases of Inherited Disease. Editions Scriver C, Beaudet A, Sly L, William. S, Valle D, 8th Edition, David Mc Graw Hill, N.Y., 8th Edition, Chapter 134, 3371-89.

• Moser H, Linke T, Fensom AH, Levade T, Sandhoff K (2001) Acid ceramidase deficiency : Farber Lipogranulomatosis, in: The Metabolic and Molecular Basis of Inherited Disease. Editions Scriver C, Beaudet A, Sly L, William. S, Valle D, 8th Edition, David Mc Graw Hill, N.Y., 8th Edition, Chapter 143, 3573-89.

• Linke T, Wilkening G, Sadeghlar F, Moczall H, Bernardo K, Schuchman E, Sandhoff K (2001) Interfacial regulation of acid ceramidase activity: Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins. J Biol Chem, 276, 5760-8.

• Kawai H, Allende ML, Wada R, Kono M, Sango K, Deng C, Miyakawa T, Crawley JN, Werth N, Bierfreund U, Sandhoff K, Proia RL (2001) Mice expressing only monosiaganglioside GM3 exhibit sudden death and audiogenic seizures. J Biol Chem, 276, 6885-8.

• Bär J, Linke T, Ferlinz K, Neumann U, Schuchman EH, Sandhoff K (2001) Molecular analysis of acid ceramidase deficiency in patients with Farber Disease. Hum Mutat, 17, 199-209.

• Linke T, Wilkening G, Lansmann S, Moczall H, Bartelsen O, Weisgerber J, Sandhoff K, (2001) Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins. Biol Chem, 382, 283-90.

• Werth N, Schuette C, Wilkening G, Lemm T, Sandhoff K (2001) Degradation of membrane-bound ganglioside GM2 by Hexosaminidase A : Stimulation by GM2 activator protein and lysosomal lipids. J Biol Chem, 276 12685-90.

• Kolter T, Sandhoff K in: B Fraser-Reid, K Tatsuta, J Thiem (2001) Animal Glycolipids: Biosynthesis and degradation. Glycoscience: Chemistry and Chemical Biology III, Chapter 7.4, 2149 - 2182, Springer, Berlin.

• Panignone S, Bergomas R, Fontanella E, Prinetti A, Sandhoff K, Grabowski GA, Delia D (2001) Up-regulation of prosaposin by the retinoid HPR and the effect on ceramide production and integrin receptors. FASEB J, 15, 1475-7.

• Schütte CG, Weisgerber J, Sandhoff K (2001) Complete analysis of the glycosylation and disulfide bond pattern of human b-hexosaminidase B by MALDI-MS. Glycobiology, 11, 549-56.

• Ferlinz K, Kopal G, Bernardo K, Linke T, Breiden B, Neumann U, Kettenhofen R, Lang F, Schuchman EH, Sandhoff K (2001) Human acid ceramidase: Processing, glycosylation and lysosomal targeting. J Biol Chem, 276, 35352-60.

• Schuette C, Pierstorff B, Huettler, S, Sandhoff K (2001) Sphingolipid activator proteins: proteins with complex functions in lipid degradation and skin biogenesis. Glycobiology, 11, 81R-90R.

• Peter-Katalinic J, Sandhoff K (2001) Highlight: Glycobiology. Biol Chem, 382, 141-2.

• Grassmé H, Jeckle A, Riehle A, Schwarz H, Berger J, Sandhoff K, Kolesnik R, Gulbins E (2001) CD95 Signaling via ceramide rich membrane rafts. J Biol Chem, 276, 12685-90.

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• Liu Y, Wada R, Kawai H, Sango K, Deng C, Tai T, McDonald MP, Araujo K, Crawley JN, Bierfreund U, Sandhoff K, Suzuki K, Proia RL (1999) A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder. J Clin Invest, 103, 497-505.

• Sakuraba H, Itoh K, Shimmoto M, Utsumi K, Kase R, Hashimoto Y, Ozawa T, Ohwada Y, Imataka G, Eguchi M, Furukawa T, Schepers U, Sandhoff K (1999) GM2 gangliosidosis AB variant - Clinical and biochemical studies of a Japanese patient. Neurology, 52, 372-7.

• Doering T, Proia RL, Sandhoff K (1999) Accumulation of protein-bound epidermal glucosylceramides in ß-glucocerebrosidase deficient type 2 Gaucher mice. FEBS Lett, 447, 167-70.

• Doering T, Holleran WM, Potratz A, Vielhaber G, Elias PM, Suzuki K, Sandhoff K (1999) Sphingolipid activator proteins are required for epidermal permeability barrier formation. J Biol Chem, 274, 11038-45.

• Utku N, Bulwin GC, Beinke S, Heinemann T, Beato F, Randall J, Schnieders B, Sandhoff K, Volk HD, Milford E, Gullans SR (1999) The human homolog of Drosophila cornichon protein is differentially expressed in alloactivated T-cells 1. Biochim Biophys Acta, 1449, 203-10.

• Giehl A, Lemm T, Bartelsen O, Sandhoff K, Blume A (1999) Interaction of the GM2-activator protein with phospholipid/ganglioside bilayer membranes and with monolayers at the air/water interface. Eur J Biochem, 261, 650-58.

• Langmann T, Buechler C, Ries S, Schaeffler A, Aslanidis C, Schuierer M, Weiler M, Sandhoff K, de Jong PJ, Schmitz G (1999) Transcription factors Sp1 and AP-2 mediate induction of acid sphingomyelinase during monocytic differentiation. J Lipid Res, 40, 870-80.

• Heinemann T, Bulwin GC, Randall J, Schnieders B, Sandhoff K, Volk HD, Milford E, Gullans SR, Utku N (1999) Genomic organization of the gene coding for TIRC7, a novel Membrane protein essential for T cell activation. Genomics, 57, 398-406.

• van Echten-Deckert G, Sandhoff K (1999) Organization and topology of sphingolipid metabolism. Comprehensive Natural Product Chemistry, Chapter 3.05, 87-106.

• Kolter T, Sandhoff K (1999) Sphingolipide-Stoffwechselwege und die Pathobiochemie neurodegenerativer Erkrankungen. Angew Chem, 111, 1632-70.

• Kolter T, Sandhoff K (1999) Sphingolipids -Their Metabolic Pathways and the Pathobiochemistry of Neurodegenerative Diseases. Angew Chem Int Ed, 38, 1532-68.

• Möbius W, Herzog V, Sandhoff K, Schwarzmann G (1999) Intracellular distribution of a biotin-labeled ganglioside, GM1, by immunoelectron microscopy after endocytosis in fibroblasts. J Histochem Cytochem, 47, 1005-14.

• Yamashita T, Wada R, Sasaki T, Deng C, Bierfreund U, Sandhoff K, Proia RL (1999) A vital role of glycosphingolipid synthesis during development and differentiation. Proc Natl Acad Sci U S A, 96, 9142-7.

• Kolter T, Doering T, Wilkening G, Werth N, Sandhoff K (1999) Recent advances in the biochemistry of glycosphingolipid metabolism. Biochem Soc Trans, 27, 409-15.

• Reichelt J, Doering T, Schnetz E, Fartasch M, Sandhoff K, Magin TM (1999) Normal ultrastructure, but altered stratum corneum lipid and protein composition in a mouse model for epidermolytic hyperkeratosis. J Invest Dermatol 113, 329-34.

• Schuette C, Doering T, Kolter T, Sandhoff K (1999) The Glycosphingolipidoses - from Disease to basic Principles of Metabolism. Biol Chem, 380, 759-66.

• Schuette C, Doering T, Kolter T, Sandhoff K (1999) Die Glykosphingolipidosen - von Krankheiten zu Prinzipien des Stoffwechsels. BIOspektrum, 5, 365-70.

• Kolter T, Sandhoff K (1999) Sphingolipidosen in "Handbuch der Molekularen Medizin". Bd. 6, 1999, 195-234.

• Ferlinz K, Linke T, Bartelsen O, Weiler M, Sandhoff K (1999) Stimulation of lysosomal sphingomyelin and ceramide degradation by sphingolipid activtor proteins. Chem Phys Lipids 102, 35-43.

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• van Echten-Deckert G, Giannis A, Schwarz A, Futerman AH, Sandhoff K (1998) 1-Methylthiodihydroceramide, a novel analog of dihydroceramide, stimulates sphinganine degradation resulting in decreased de novo sphingolipid biosynthesis. J Biol Chem, 273, 1184-91.

• Kolter T, Sandhoff K (1998) Recent advances in the biochemistry of sphingolipidoses. Brain Pathol, 8, 79-100.

• Breen KC, Potratz A, Georgopoulou N, Sandhoff K (1998) The generation and characterization of a rat neural cell line overexpressing the a2,6(N)sialyltransferase. Glycoconj J, 15, 199-202.

• Brenner B, Ferlinz K, Grassmé H, Weller M, Koppenhoefer U, Dichgans J, Sandhoff K, Lang F, Gulbins E (1998) Fas/CD95/Apo-I activates the acidic sphingomyelinase via caspases. Cell Death Different, 5, 29-37.

• Liu Y, Suzuki K, Reed JD, Grinberg A, Westphal H, Hoffmann A, Döring T, Sandhoff K, Proia RL (1998) Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure (SIMP). Proc Natl Acad Sci U S A, 95, 2503-8.

• Schütte CG, Lemm T, Glombitza GJ, Sandhoff K (1998) Complete localization of disulfide bonds in GM2 activator protein. Protein Sci 7, 1039-45.

• Norflus F, Tifft CJ, McDonald MP, Goldstein G, Crawley JN, Hoffmann A, Sandhoff K, Suzuki K, Proia RL (1998) Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. J Clin Invest 101, 1881-8.

• Kolter T, Sandhoff K (1998) Glycolipids: Structure, function and metabolism. In: Glycobiology Catalog 1998 (Calbiochem) 132-3.

• Sandhoff K, Kolter T, van Echten-Deckert G (1998) Sphingolipid activator proteins and the pathology of the cells. in: "Sphingolipids as Signaling Modulators in the Nervous System" (Ledeen, Robert, W.; Hakomori, Sen-Itiroh; Yates, Allan, J.; Schneider, Jay S.; Yu, Robert, K. eds), New York Academy of Sciences "Annals" Vol. 845, 139-51.

• Li CM, Hong SB, Kopal G, He X, Linke T, Hou WS, Koch J, Gatt S, Sandhoff K, Schuchman EH (1998) Cloning and characterization of the full-length cDNA and genomic sequences encoding murine acid ceramidase - Analysis of mRNA and enzymatic expression in adult mice. Genomics 50, 267-74.

• Sandhoff K, Kolter T (1998) Processing of sphingolipid activator proteins and the topology of lysosomal digestion. Acta Biochim Pol, 45, 373-84.

• Kolter T, Sandhoff K (1998) Glycosphingolipid degradation and animal models of GM2-gangliosidoses. J Inher Metab Dis, 21, 548-63.

• Hiesberger T, Hüttler S, Rohlmann A, Schneider W, Sandhoff K, Herz J (1998) Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP). EMBO J, 17, 4617-25.

• Landrieu P, Blanche S, Vanier MT, Metral S, Husson B, Sandhoff K, Fischer A (1998) Bone marrow transplantation in metachromatic leukodystrophy caused by saposin-B deficiency: A case report with a 3-year follow-up period. J Pediatr, 133, 129-32.

• Sandhoff K (1998) Hemmnisse universitärer Forschung, in: Zwei Jahrhunderte Wissenschaft und Forschung in Deutschland - Entwicklungen - Perspektiven (Symposium aus Anlaß des 175jährigen Bestehens der Gesellschaft Deutscher Naturforscher und Ärzte (GDNÄ) 19. - 20. September 1997 in Lübeck) (Dietrich von Engelhard, ed.) Wissenschaftliche Verlagsgesellschaft mbH Stuttgart, 256-9.

• Bartelsen O, Lansmann S, Nettersheim M, Lemm T, Ferlinz K, Sandhoff K (1998) Expression of recombinant human acid sphingomyelinase in insect Sf21 cells: Purification, processing and enzymatic characterization. J Biotechnol, 63, 29-40.

• Uhlhorn-Dierks G, Kolter T, Sandhoff K (1998) Wie werden Schwefelsäureester in der Natur gespalten? Eine neuartige posttranslationale Proteinmodifikation bei Sulfatasen. Angew Chem, 110, 2591-4.

• Uhlhorn-Dierks G, Kolter T, Sandhoff K (1998) How does nature cleave sulfuric acid esters? A novel posttranslational modification of sulfatases. Angew Chem Int Ed, 37, 18, 2453-5.

• Wilkening G, Linke T, Sandhoff K (1998) Lysosomal degradation on vesicular membrane surfaces: Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators. J Biol Chem, 273, 30271-8.

• Sandhoff K, Kolter T (1998) Enzymology of lysosomal glycolipid catabolism. Tends Glycosci Glyc, 10, 455-68.

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• Ferlinz K, Hurwitz R, Moczall H, Lansmann S, Schuchman EH, Sandhoff K (1997) Functional characterization of the N-glycosylation sites of human acid sphingomyelinase by site-directed mutagenesis. Eur J Biochem, 243, 511-7.

• Glombitza GJ, Becker E, Kaiser HW, Sandhoff K (1997) Biosynthesis, processing and intracellular transport of GM2 activtor protein in human epidermal keratinozytes: the lysosomal targeting of the GM2 activator is independent of a mannose-6-phosphate signal. J Biol Chem, 272, 5199-207.

• Pennybacker M, Schuette CG, Liessem B, Hepbildikler ST, Kopetka JA, Ellis MR, Myerowitz R, Sandhoff K, Proia RL (1997) Evidence for the involvement of Glu-355 in the catalytic action of human ß-hexosaminidase B. J Biol Chem, 272, 8002-6.

• Burkhardt JK, Hüttler S, Klein A, Möbius W, Habermann A, Griffiths G, Sandhoff K (1997) Accumulation of sphingolipids in SAP-precursor (prosaposin) deficient fibroblasts occurs within lysosomes and can be completely reversed by treatment with human SAP-precursor. Eur J Cell Biol, 73, 10-8.

• Henseler M, Harzer K, Sandhoff K (1997) Sphingolipid activator protein. in: The Metabolic & Molecular Bases of Inherited Disease CD-ROM (eds. Scriver, Charles, R., Beaudet, Arthur, L., Sly, William, S. and Valle, David) 7th Edition, David Mc. Graw Hill, N.Y., Addendum Chapter 76

• Schick A, Schwarzmann G, Kolter T, Sandhoff K (1997) Synthesis of tritium labelled phosphate analogues of sphinganine-1-phosphate. J Label Comp Radiopharm. 39, 441-51.

• Albrecht B, Pohlentz G, Sandhoff K, Schwarzmann G (1997) Synthesis and mass spectrometric characterization of digoxingenin and biotin labeled ganglioside GM1 and their uptake by and metabolism in cultured cells. Chem Phys Lipids, 86, 37-50.

• van Echten-Deckert G, Zschoche A, Bär T, Schmidt RR, Raths A, Heinemann T, Sandhoff K (1997) cis-4-Methylsphingosine decreases sphingolipid biosynthesis by specifically interfering with serine palmitoyltransferase activity in primary cultured neurons. J Biol Chem, 272, 15825-33.

• Wieder T, Geilen CC, Kolter T, Sadeghlar F, Sandhoff K, Brossmer R, Ihrig P, Perry D, Orfanos CE, Hannun YA. (1997) Bcl-2 antagonizes apototic cell death induced by two new ceramide analogues. FEBS Lett, 411, 260-4.

• Liu Y, Hoffmann A, Grinberg A, Westphal H, McDonald MP, Miller KM, Crawley JN, Sandhoff K, Suzuki K, Proia RL (1997) Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment. Proc Natl Acad Sci U S A, 94, 8138-43.

• Michel C, van Echten-Deckert G, Rother J, Sandhoff K, Wang E, Merrill AH Jr (1997) Characterization of ceramide synthesis - A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide. J Biol Chem 272, 22432-7.

• Kolter T, Sandhoff K (1997) Sialic acids - why always a-linked? Glycobiology, 7, VII-IX.

• Grassmé H, Gulbins E, Brenner B, Ferlinz K, Sandhoff K, Harzer K, Lang F, Meyer TF (1997) Acid sphingomyelinase mediates entry of N. gonorrhoeae into non-phagocytic cells. Cell, 91, 605-6.

• Sandhoff K, Kolter T (1997) Biochemistry of glycosphingolipid degradation. Clin Chim Acta, 266, 51-61.

• van Echten-Deckert G, Klein A, Linke T, Heinemann T, Weisgerber J, Sandhoff K (1997) Turnover of endogenous ceramide in cultured normal and Farber fibroblasts. J Lipid Res, 38, 2569-79.

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• Henseler M, Klein A, Reber M, Vanier MT, Landrieu P, Sandhoff K (1996) Analysis of a splice-site mutation in the sap-precursor gene of a patient with metachromatic leukodystrophy. Am J Hum Genet, 58, 65-74.

• Schick A, Kolter T, Giannis A, Sandhoff K (1996) Synthesis of phosphoramide analogues of sphinganine-1-phosphate. Tetrahedron 52, 2945-56.

• Sandhoff K, Kolter T (1996) Topology of glycosphingolipid degradation. Trends Cell Biol, 6, 98-103. (Cover)

• Henseler M, Klein A, Glombitza GJ, Suzuki K, Sandhoff K (1996) 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. J Biol Chem, 271, 8416-23.

• Zacharias C, van Echten-Deckert G, Wang E, Merrill AH Jr, Sandhoff K (1996) The effect of fumonisin B1 on developing chick embryos: correlation between de novo sphingolipid biosynthesis and gross morphological changes. Glycoconjugate J, 13, 167-75.

• Fujita N, Suzuki K, Vanier MT, Popko B, Maeda N, Klein A, Henseler M, Sandhoff K, Nakayasu H, Suzuki K (1996) Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids. Hum Mol Genet, 5, 711-25.

• Sandhoff K (1996) Glykolipide der Zelloberfläche und die Pathobiochemie der Zelle. in Vorträge N 422 Nordrhein-Westfälische Akademie der Wissenschaften, 402. Sitzung am 1. Juli 1994 in Düsseldorf, Westdeutscher Verlag.

• Pennybacker M, Liessem B, Moczall H, Tifft CJ, Sandhoff K, Proia RL (1996) Identification of domains in human ß-hexosaminidase that determine substrate specificity. J Biol Chem, 271, 17377-82.

• Arni S, Ilangumaran S, van Echten-Deckert G, Sandhoff K, Poincelet M, Briol A, Rungger-Brändle E, Hoessli DC (1996) Differential regulation of Src-family protein tyrosine kinases in GPI domains of T lymphocyte plasma membranes. Biochem Biophys Res Commun, 225, 801-7.

• Kase R, Bierfreund U, Klein A, Kolter T, Itoh K, Suzuki M, Hashimoto Y, Sandhoff K, Sakuraba H (1996) Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal alpha-galactosidase in a detergent-free liposomal system. FEBS Lett, 393, 74-6.

• Müller E, Giehl A, Schwarzmann G, Sandhoff K, Blume A (1996) Oriented 1,2-Dimyristoyl-sn-glycero-3-phosphorylcholine/ganglioside membranes. A fourier transform infrared attenuated total reflection spectroscopic study. Band assignments; orientational, hydrational, and phase behaviour; and effects of Ca2+ binding. Biophys J, 71, 1400-21.

• Sango K, McDonald MP, Crawley JN, Mack ML, Tifft CJ, Skop E, Starr CM, Hoffmann A, Sandhoff K, Suzuki K, Proia RL (1996) Mice lacking both subunits of lysosomal ß-hexosaminidase diplay gangliosidosis and mucolipidosis. Nat Genet, 14, 348-52.

• Schepers U, Glombitza G, Lemm T, Hoffmann A, Chabas A, Ozand P, Sandhoff K (1996) Molecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variant. Am J Hum Genet, 59, 1048-56.

• Koch J, Gärtner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K (1996) Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase - Identification of the first molecular lesion causing Farber disease. J Biol Chem, 271, 33110-5.

• Lansmann S, Ferlinz K, Hurwitz R, Glombitza G, Sandhoff K (1996) Purification of the acid sphingomyelinase from human placenta: Characterization and N-terminal sequence. FEBS Lett, 399, 227-31.

• Vielhaber G, Hurwitz R, Sandhoff K (1996) Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP) precursor in cultured human fibroblasts - Mannose 6-phosphate receptor-independent endocytosis of SAP precursor. J Biol Chem, 271, 32438-46.

• Kolter T, Sandhoff K (1996) Inhibitors of glycosphingo-lipid biosynthesis. Chem Soc Rev, 25, 371-81.

• Akli S, Guidotti JE, Vigne E, Perricaudet M, Sandhoff K, Kahn A, Poenaru L (1996) Restoration of hexosaminidase A activity in human Tay-Sachs fibroblats via adenoviral vector-mediated gene transfer. Gene Ther, 9, 769-74.

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• Sandhoff K, Harzer K, Fürst W (1995) Sphingolipid activator proteins. The Metabolic and Molecular Bases of Inherited Disease, eds. Scriver, Charles, R., Beaudet, Arthur, L., Sly, William, S., and Valle, David, 7th Edition, David Mc. Graw Hill, N.Y., Chapter 76, 2427-41.

• Gravel RA, Clarke JTR, Kaback MM, Mahuran D, Sandhoff K, Suzuki K (1995) The GM2-gangliosidosis. The Metabolic and Molecular Bases of Inherited Disease, eds. Scriver, Charles, Beaudet, Arthur, L., Sly, William, S., and Valle, David. 7th Edition, David Mc Graw Hill, N.Y., Chapter 92, 2839-79.

• Sandhoff K, van Echten-Deckert, G (1995) Glycolipids of cellular surfaces: Topology of metabolism, function and pathobiochemistry of glycolipid binding proteins. Trafficking of Intracellular Membranes: from molecular sorting to membrane fusion (Pedroso de Lima, M. C., Düzgünes, N. and Hoekstra, D.), NATO ASI Series, Springer Verlag, Vol. H 91, 235-46.

• Ferlinz K, Sandhoff K (1995) Niemann-Pick disease type A and B - Natural history of lysosomal shingomyelinase. Trafficking of intracellular membranes: from molecular sorting to membrane fusion (Pedroso de Lima, M. C., Düzgünes, N. and Hoekstra, D.), NATO ASI Series, Springer Verlag, Vol. H 91, 247-63.

• Bernardo K, Hurwitz R, Zenk T, Desnick RJ, Ferlinz K, Schuchman EH, Sandhoff K (1995) Purification, characterization and biosynthesis of human acid ceramidase. J Biol Chem, 270, 11098-102.

• Tyynelä J, Baumann M, Henseler M, Sandhoff K, Haltia M (1995) Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: An immunological study. Acta Neuropath, 89, 391-98.

• Ferlinz K, Hurwitz R, Weiler M, Suzuki K, Sandhoff K, Vanier MT (1995) Molecular analyis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease. Am J Hum Genet, 56, 1343-9.

• Suzuki K, van Echten-Deckert G, Klein A, Sandhoff K (1995) Glycosphingolipids and sphingolipid activator proteins: Cell biology, biochemistry and molecular genetics. In: Biochemistry of Cell Membranes. A compendium of selected topics" (eds. Sergio Papa and Joseph M. Tager), 137-49.

• Tyynela J, Baumann M, Henseler M, Sandhoff K, Haltia M (1995) Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL). Am J Med Genet, 57, 294-7.

• Horinouchi K, Erlich S, Perl DP, Ferlinz K, Bisgaier CL, Sandhoff K, Desnick RJ, Stewart CL, Schuchman EH (1995) Acid sphingomyelinase deficient mice: A new model for the study of types A and B Niemann-Pick disease. Nat Genet, 10, 288-93.

• Liessem B, Glombitza GJ, Knoll F, Lehmann J, Kellermann J, Lottspeich F, Sandhoff K (1995) Photoaffinity labeling of human lysosomal ß-hexosaminidase B - Identification of Glu-355 at the substrate binding site. J Biol Chem, 270, 23693-9.

• Sango K, Yamanaka S, Hoffmann A, Okuda Y, Grinberg A, Westphal H, McDonald MP, Crawley JN, Sandhoff K, Suzuki K, Proia RL (1995) Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype pathology and ganglioside metabolism. Nat Genet, 11, 170-6.

• Schick A, Kolter T, Giannis A, Sandhoff K (1995) Synthesis of phosphonate analogues of sphinganine-1-phosphate and sphingosine-1-phosphate. Tetrahedron, 51, 11207-18.

• Sandhoff K, Kolter T (1995) Glykolipide der Zelloberfläche - Biochemie ihres Abbaus. Naturwissenschaften, 82, 403-13.

• Kolter T, Sandhoff K (1995) Neue Gehirnlipide mit schlafinduzierender Wirkung. Angew Chem, 107, 2559-60.

• Kolter T, Sandhoff K (1995) New brain lipids that induce sleep. Angew Chem Int Ed Engl 34, 2363-4.

• Sandhoff K (1995) Die Aufklärung zentralnervöser Speicherkrankheiten und die Entdeckung der Sphingolipid-Aktivatorproteine. MPI-Spiegel, 6, 69-70.

• Haltia M, Tyynelä J, Baumann M, Henseler M, Sandhoff K (1995) Immunological studies on sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses. Gerontology, 41, Suppl. 2, 239-46.

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• Sandhoff K, van Echten G (1994) Ganglioside metabolism: enzymology, topology and regulation, in: Progress in Brain Research" L. Svennerholm, A.K. Ashbury, R.A. Reisfeld, K. Sandhoff, K. Suzuki, G. Tettamanti, and G. Toffano, eds., Elsevier Science B.V., Chapter 2, Vol. 101, 17-29

• Hurwitz R, Ferlinz K, Vielhaber G, Moczall H, Sandhoff K (1994) Processing of human acid sphingomyelinase in normal and I-cell fibroblasts. J Biol Chem, 269, 5440-5.

• Sandhoff K, van Echten G (1994) Metabolism of gangliosides - Topology, pathobiochemistry an sphingolipid activator proteins: in "Current Topics in Membranes" (Hoekstra, Dick ed.), Chapter 3, Vol. 40, p. 75-91.

• Zacharias C, van Echten-Deckert G, Plewe M, Schmidt RR, Sandhoff K (1994) A truncated epoxy-glucosylceramide uncouples glycosphingolipid biosynthesis by decreasing lactosylceramide synthase activity. J Biol Chem, 269, 13313-7.

• Klein A, Henseler M, Klein C, Suzuki K, Harzer K, Sandhoff K (1994) Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo. Biochem Biophys Res Commun, 200, 1440-8.

• Zschoche A, Fürst W, Schwarzmann G, Sandhoff K (1994) Hydrolysis of lactosylceramide by human galactosylceramidase and GM1-ß-galactosidase in a detergent-free system and its stimulation by sphingolipid activator proteins, sap-B and sap-C. Eur J Biochem, 222, 83-90.

• Sandhoff K, Klein A (1994) Intracellular trafficking of glycosphingolipids.Role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion. FEBS Lett, 346, 103-7.

• Ferlinz K, Hurwitz R, Vielhaber G, Suzuki K, Sandhoff K (1994) Occurence of two molecular forms of human acid sphingomyelinase. Biochem J, 301, 855-62.

• Hurwitz R, Ferlinz K, Sandhoff K (1994) The tricyclic antidepressant desipramine causes proteolytic degradation of lysosomal sphingomyelinase in human fibroblasts. Biol Chem Hoppe-Seyler, 375, 447-50.

• Sandhoff K, van Echten G (1994) Topology and regulation of ganglioside metabolism - Function and pathobiochemistry of sphingolipid activator proteins. In: Glyco- and Cellbiology -44. Colloquium Mosbach - (Wieland, F., Reutter, W. eds.), 69 -79.

• Kolter T, van Echten-Deckert G, Sandhoff K (1994) Synthesis of sphinganine analogues modified in the head group. Tetrahedron 50, 13425-32.

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