Jordans, S., Hardt, R., Becker, I., Winter, D., Wang-Eckhardt, L., and Eckhardt, M. (2022): Age-dependent increase in Schmidt-Lanterman incisures and a Cadm4 associated membrane skeletal complex in fatty acid 2-hydroxylase deficient mice, a mouse model of spastic paraplegia SPG35. Mol. Neurobiol. 59:3969-3979
Wang-Eckhardt, L., Sylvester, M., Becker, I., Allam, J.-P., and Eckhardt, M. (2022): Citrylglutamate synthase deficient male mice are subfertile with impaired histone and transition protein 2 removal in late spermatids. Biochem. J. 479:953-972
Wang-Eckhardt, L., Becker, I., Wang, Y., Yuan, J., and Eckhardt, M. (2022): Absence of endogenous carnosine synthesis does not increase protein carbonylation and advanced lipoxidation end products in brain, kidney or muscle. Amino Acids 54:1013-1023
2021
Wang-Eckhardt, L., Becker, I., and Eckhardt, M. (2021): The PGRMC1 antagonist AG-205 inhibits synthesis of galactosylceramide and sulfatide. Cells 10:3520
Qi, L., Martin-Sandoval, M.S., Mathews, T.P., Gu, W., Eckhardt, M., Zhao, Z., Agathocleous, M., and Morrison, S.J. (2021): Aspartate is limiting for hematopoietic stem cell function. Cell Stem Cell 28:1982-1999
Becker, I., Wang-Eckhardt, L., Lodder-Gadaczek, J., Wang, Y. Grünewald, A., and Eckhardt, M. (2021) Mice deficient in the NAAG synthetase II gene Rimkla are impaired in a novel object recognition task. J. Neurochem. 157:2008-2023
2020
Hardt, R., Jordans, S., Winter, D., Gieselmann, V., Wang-Eckhardt, L., and Eckhardt, M. (2020): Decreased turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35. Hum. Mol. Genet. 29:3616-3630
Wang-Eckhardt, L., Bastian, A., Bruegmann, T., Sasse, P., and Eckhardt, M. (2020): Carnosine synthase deficiency is compatible with normal skeletal muscle and olfactory function but causes reduced olfactory sensitivity in aging mice. J. Biol. Chem. 295:17100-17113
Wang-Eckhardt, L., and Eckhardt M. (2020): A progesterone receptor membrane component 1 antagonist induces large vesicles independent of progesterone receptor membrane component 1 expression. Biol. Chem. 401:1093-1099
Li, L., Pu, Q., Hintze, M., Wang, Y., Eckhardt, M., Gieselmann, V., Tiemann, I., Qi, X., Cai, D., Wang, J., and Huang, R. (2020): BDNF and NGF signals originating from sensory ganglia promote cranial motor axon growth. Exp. Brain Res. 238:111-119
Nagarajan, B., Harder, A., Japp, A., Häberlein, F., Mingardo, E., Kleinert, H., Yilmaz, Ö., Zoons, A., Rau, B., Christ, A., Kubitscheck, U., Eiberger, B., Sandhoff, R., Eckhardt, M., Hartmann, D., and Odermatt, B. (2020): CNS myelin protein 36K regulates oligodendrocyte differentiation through Notch. Glia 68:509-527
2019
Hofer, D.C., Zirkovits, G., Pelzmann, H.J., Huber, K., Pessentheiner, A.R., Xia, W., Uno, K., Miyazaki, T., Kon, K., Tsuneki, H., Pendl, T., Al Zoughbi, W., Madreiter-Sokolowski, C.T., Trausinger, G., Abdellatif, M., Schoiswohl, G., Schreiber, R., Eisenberg, T., Magnes, C., Sedej, S., Eckhardt, M., Sasahara, M., Sasaoka, T., Nitta, A., Hoefler, G., Graier, W.F., Kratky, D., Auwerx, J., and Bogner-Strauss, J.G. (2019): N-acetylaspartate availability is essential for juvenile survival on fat-free diet and determines metabolic health. FASEB J. 33:13808-13824
Fewou, S.N., Röckle, I., Hildebrandt, H., and Eckhardt, M. (2019): Transgenic overexpression of polysialyltransferase ST8SiaIV under the control of a neuron-specific promoter does not affect brain development but impairs exploratory behavior. Glycobiology 29:657-668
2010 - 2018
Hardt, R., Winter, D., Gieselmann, V., and Eckhardt, M. (2018): Identification of progesterone receptor membrane component-1 as an interaction partner and possible regulator of fatty acid 2-hydroxylase. Biochem. J. 475:853-871
von Gerichten, J., Schlosser, K., Lamprecht, D., Morace, I., Eckhardt, M., Wachten, D., Jennemann, R., Gröne, H.-J., Mack, M., and Sandhoff, R. (2017): Diastereomer-specific quantification of bioactive hexosylceramides from bacteria and mammals. J. Lipid Res. 58:1247-1258
Eckhardt, M., and Wang-Eckhardt, L. (2015): A commercial human protamine-2 antibody used in several studies to detect mouse protamine-2 recognizes mouse transition protein-2 but not protamine-2. Mol. Hum. Reprod. 21:825-831
Maier, H., Wang-Eckhardt, L., Hartmann, D., Gieselmann, V., and Eckhardt, M. (2015): N-acetylaspartate synthase deficiency corrects the myelin phenotype in a Canavan disease mouse model but does not affect survival time. J. Neurosci. 35:14501-14516
Doerr, J., Böckenhoff, A., Ewald, B., Ladewig, J., Eckhardt, M., Gieselmann, V., Matzner, U., Brüstle, O., and Koch, P. (2015): Arylsulfatase A overexpressing human iPSC-derived neural cells reduce CNS sulfatide storage in a mouse model of metachromatic leukodystrophy. Mol. Ther. 23:1519-1531
Ebel, P., Imgrund, S., Vom Dorp, K., Hofmann, K., Maier, H., Drake, H., Degen, J., Dörmann, P., Eckhardt, M., Franz, T., and Willecke, K. (2014): Ceramide Synthase 4 deficiency in mice causes lipid alterations in sebum and results in alopecia. Biochem. J. 461:147-158
Ebel, P., vom Dorp, K., Petrasch-Parwez, E., Zlomuzica, A., Kinugawa, K., Mariani, J., Minich, D., Ginkel, C., Degen, J., Eckhardt, M., Dere, E., Dörmann, P., and Willecke, K. (2013): Inactivation of ceramide synthase 6 in mice results in an altered sphingolipid metabolism and behavioral abnormalities. J. Biol. Chem. 288:21433-21447
Lodder-Gadaczek, J., Gieselmann, V., and Eckhardt, M. (2013): Vesicular uptake of N-acetylaspartylglutamate is catalyzed by sialin (SLC17A5). Biochem. J. 454:31-38
Bakhti, M., Snaidero, N., Schneider, D., Aggarwal, S., Möbius, W., Janshoff, A., Eckhardt, M., Nave, K.A., and Simons, M. (2013): Loss of electrostatic cell-surface repulsion mediates myelin membrane adhesion and compaction in the central nervous system. Proc. Natl. Acad. Sci. USA 110:3143-3148
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., and 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. 287:41888-41902
Matthes, F., Stroobants, S., Gerlach, D., Wohlenberg, C., Wessig, C., Fogh, J., Gieselmann, V., Eckhardt, M., D'Hooge, R., and Matzner, U. (2012): Efficacy of enzyme replacement therapy in an aggravated mouse model of metachromatic leukodystrophy declines with age. Hum. Mol. Genet. 21:2599-2609
Kuronen, M., Hermansson, M., Manninen, O., Zech, I., Talvitie, M., Laitinen, T., Gröhn, O., Somerharju, P., Eckhardt, M., Cooper, J., Lehesjoki, A.-E., Lahtinen, U., and Kopra, O. (2012): Galactolipid deficiency characterizes the early pathogenesis of the neuronal ceroid lipofuscinosis model Cln8mnd: implications to delayed myelination and oligodendrocyte maturation. Neuropathol. Appl.Neurobiol. 38:471-486
Jungnickel, J., Eckhardt, M., Haastert, K., Claus, P., Bronzlik, P., Lipokatic-Takacs, E., Maier, H., Gieselmann, V., and Grothe, C. (2012): Schwann cell-derived polysialic acid mediates different effects during peripheral nerve regeneration. Glycobiology 22:107-115
Maier, H., Meixner, M., Hartmann, D., Sandhoff, R., Wang-Eckhardt, L., Zöller, I., Gieselmann, V., and Eckhardt, M. (2011): Normal fur development and sebum production depends on fatty acid 2-hydroxylase expression in sebaceous glands. J. Biol. Chem. 286:25922-25934
Marsching, C., Eckhardt, M., Gröne, H.-J., Sandhoff, R., and Hopf, C. (2011): Imaging of complex sulfatides SM3 and SB1a in mouse kidney using MALDI-TOF/TOF mass spectrometry. Anal. Bioanal. Chem. 401:53-64
Faldini, E., Stroobants, S., Lüllmann-Rauch, R., Eckhardt, M., Gieselmann, V., Balschun, D., and D’Hooge, R. (2011): Telencephalic histopathology and changes in behavioural and neural plasticity in a murine model for metachromatic leukodystrophy. Behav. Brain Res. 222:309-314
Lodder-Gadaczek, J., Becker, I., Gieselmann, V., Wang-Eckhardt, L., and Eckhardt, M. (2011): N-acetylaspartylglutamate synthetase-II synthesizes N-acetylaspartyl-glutamylglutamate. J. Biol. Chem. 286:16693-16706
Meixner, M., Jungnickel, J., Grothe, C., Gieselmann, V., and Eckhardt, M. (2011): Myelination in the absence of UDP-galactose:ceramide galactosyltransferase and fatty acid 2-hydroxylase. BMC Neurosci. 12:22
Henseler, D., Turner, J., Eckhardt, M., van der Mark, M., Revsin, Y., Lin, M., Kranz, T., and Meyer, J. (2011): Unusual 5'-regulatory structure and regulation of the murine Mlc1 gene: lack of promoter-specific functional elements. J. Nucleic Acids Investig. 2:70-76 (e11)
Eckhardt, M. (2010): Pathology and current treatment of neurodegenerative sphingolipidoses. Neuromolecular Med. 12:362-382
Becker, I., Lodder, J., Gieselmann, V., and Eckhardt, M. (2010): Molecular characterization of N-acetylaspartylglutamate synthetase. J. Biol. Chem. 285:29156-29164
Dick, K.J., Eckhardt, M., Paisán-Ruiz, C., Alkhayat Alshehhi, A., Proukakis, C., Sibtain, N., Maier, H., Sharifi, R., Patton, M.A., Bashir, W., Koul, R., Raeburn, S.A., Gieselmann, V., Houlden, H., and Crosby, A.H. (2010): Mutation of fatty acid 2-hydroxylase underlies a complicated form of hereditary spastic paraplegia (SPG35). Hum. Mutat. 31:E1251-E1260
van Zyl, R., Gieselmann, V., and Eckhardt, M. (2010): Elevated sulfatide levels in neurons causes lethal audiogenic seizures in mice. J. Neurochem. 112:282-295
2001 - 2009
Imgrund, S., Hartmann, D., Farwanah, H., Eckhardt, M., Sandhoff, R., Degen, J., Gieselmann, V., Sandhoff, K., and Willecke K. (2009): Adult ceramide synthase 2 (CerS2) deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. J. Biol. Chem. 284:33549-33560
Zöller, I., Meixner, M., Hartmann, D., Büssow, H., Meyer, R., Gieselmann, V., and Eckhardt, M. (2008): Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration. J. Neurosci. 28:9741-9754
Stroobants, S., Leroy, T., Eckhardt, M., Aerts, J.-M., Berckmans, D., and D'Hooge, R. (2008): Early signs of neurolipidosis-related behavioural alterations in a murine model of metachromatic leukodystrophy. Behav. Brain Res. 189:306-316
Eckhardt, M. (2008): The role and metabolism of sulfatide in the nervous system. Mol. Neurobiol. 37:93-103
Becker, I., Wang-Eckhardt, L., Yaghootfam, A., Gieselmann, V., and Eckhardt, M. (2008): Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2. Histochem. Cell Biol. 129:233-241
Townson, K., Greenshields, K., Veitch, J., Nicholl, D., Eckhardt, M., Galanina, O., Bovin, N., Samain, E., Antoine, T., Bundle, D., Zhang, P., Chun Ling, C., and Willison, H.J. (2007): Sulfatide binding properties of murine and human anti-ganglioside antibodies. Glycobiology 17:1156-1166
Ramakrishnan, H., Khalaj-Hedayati, K., Lüllmann-Rauch, R., Wessig, C., Fewou, S.N., Maier, H., Goebel, H.H., Gieselmann, V., and Eckhardt, M. (2007): Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy. J. Neurosci. 27:9482-9490
Eckhardt, M., Khalaj-Hedayati, K., Pitsch, J., Lüllmann-Rauch, R., Beck, H., Fewou, S.N., and Gieselmann, V. (2007): Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy. J. Neurosci. 27:9009-9021
Yaghootfam, A., Sorkalla, T., Häberlein, H., Gieselmann, V., Kappler, J., and Eckhardt, M. (2007): Cerebroside sulfotransferase forms homodimers in living cells. Biochemistry 46:9260-9269
Fewou, S.N., Ramakrishnan, H., Büssow, H., Gieselmann, V., and Eckhardt, M. (2007): Down-regulation of polysialic acid is required for efficient myelin formation. J. Biol. Chem. 282:16700-16711
Lütjohann, D., Harzer, K., Gieselmann, V., and Eckhardt, M. (2006): Reduced brain cholesterol content in arylsulfatase A-deficient mice. Biochem. Biophys. Res. Commun. 344:647-650
Tiralongo, J., Ashikov, A., Routier, F., Eckhardt, M., Bakker, H., Gerardy-Schahn, R., and von Itzstein, M. (2006): Functional expression of the CMP-sialic acid transporter in Escherichia coli and its identification as a simple mobile carrier. Glycobiology 16:73-81
Zöller, I., Büssow, H., Gieselmann, V., and Eckhardt, M. (2005): Oligodendrocyte specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level. Glia 52:190-198
Fewou, S.N., Büssow, H., Schaeren-Wiemers, N., Vanier, M.T., Macklin, W.B., Gieselmann, V., and Eckhardt, M. (2005): Reversal of non-hydroxy:α-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase. J. Neurochem. 94:469-481
Eckhardt, M., Yaghootfam, A., Fewou, S.N., Zöller, I., and Gieselmann, V. (2005): A mammalian fatty acid hydroxylase responsible for the formation of α-hydroxylated galactosylceramide in myelin. Biochem. J. 388:245-254
Yaghootfam, A., Gieselmann, V., and Eckhardt, M. (2005): Delay of myelin formation in arylsulfatase A deficient mice. Eur. J. Neurosci. 21:711-720
Eckhardt, M., Fewou, S.N., Ackermann, I., and Gieselmann, V. (2002): N-glycosylation is required for full enzymic activity of the murine galactosylceramide sulphotransferase. Biochem. J. 368:317-324
Lühn, K., Wild, M.K., Eckhardt, M., Gerardy-Schahn, R., and Vestweber, D. (2001): The defective gene in leukocyte adhesion deficiency II encodes a putative GDP-fucose transporter. Nat. Genet. 28:69-72
1994 - 2000
Windfuhr, M., Manegold, A., Mühlenhoff, M., Eckhardt, M., and Gerardy-Schahn, R. (2000): Molecular defects that cause loss of polysialic acid in the complementation group 2A10. J. Biol. Chem. 275:32861-32870
Eckhardt, M., Bukalo, O., Chazal, G., Wang, L., Goridis, C., Schachner, M., Gerardy-Schahn, R., Cremer, H., and Dityatev, A. (2000): Mice deficient in the polysialyltransferase ST8SiaIV/PST-1 allow discrimination of the roles of neural cell adhesion molecule protein and polysialic acid in neural development and synaptic plasticity. J. Neurosci. 20:5234-5244
Eckhardt, M., Barth, H., Blöcker, D., and Aktories, K. (2000): Binding of Clostridium botulinum C2 toxin to asparagine-linked complex and hybrid carbohydrates. J. Biol. Chem. 275:2328-2334
Gerardy-Schahn, R., and Eckhardt, M. (2000): Nucleotide Sugar Transporters. In: Beat Ernst, Gerald W. Hart, Pierre Sinaý (Editors): Carbohydrates in Chemistry and Biology: A Comprehensive Handbook. (Part II, 3.), Chapter 41, pp. 19-36; Wiley-VCH Verlag GmbH, Weinheim, Germany (doi: 10.1002/9783527618255.ch41); Print ISBN: 9783527295111; Online ISBN: 9783527618255
Eckhardt, M., Gotza, B., and Gerardy-Schahn, R. (1999): Membrane topology of the mammalian CMP-sialic acid transporter. J. Biol. Chem. 274:8779-8787
Eckhardt, M., and Gerardy-Schahn, R. (1998): Genomic organization of the murine polysialyltransferase gene ST8SiaIV (PST-1). Glycobiology 8:1165-1172
Münster, A.K., Eckhardt, M., Potvin, B., Mühlenhoff, M., Stanley, P., and Gerardy-Schahn, R. (1998): Mammalian cytidine 5´-monophosphate N-acetylneuraminic acid synthetase: A nuclear protein with evolutionarily conserved structural motifs. Proc. Natl. Acad. Sci. USA 95:9140-9145
Eckhardt, M., Gotza, B., and Gerardy-Schahn, R. (1998): Mutants of the CMP-sialic acid transporter causing the Lec2 phenotype. J. Biol. Chem. 273:20189-20195
Mühlenhoff, M., Eckhardt, M., and Gerardy-Schahn, R. (1998): Polysialic acid: three-dimensional structure, biosynthesis, and function. Curr. Opin. Struct. Biol. 8:558-564
Glüer, S, Schelp, C., Madry, N., von Schweinitz, D., Eckhardt, M., and Gerardy-Schahn, R. (1998): Serum polysialylated neural cell adhesion molecule in childhood neuroblastoma. Br. J. Cancer 78:106-110
Eckhardt, M., and Gerardy-Schahn, R. (1997): Molecular cloning of the hamster CMP-sialic acid transporter. Eur. J. Biochem. 248:187-192
Berninsone, P., Eckhardt, M., Gerardy-Schahn, R., and Hirschberg, C.B. (1997): Funtional expression of the murine Golgi CMP-sialic acid transporter in Saccharomyces cerevisiae. J. Biol. Chem. 272:12616-12619
Mühlenhoff, M., Eckhardt, M., Bethe, A., Frosch, M., and Gerardy-Schahn, R. (1996): Autocatalytic polysialylation of polysialyltransferase-1. EMBO J. 15:6943-6950
Mühlenhoff, M., Eckhardt, M., Bethe, A., Frosch, M., and Gerardy-Schahn, R. (1996): Polysialylation of NCAM by a single enzyme. Curr. Biol. 6:1188-1191
Eckhardt, M., Mühlenhoff, M., Bethe, A., and Gerardy-Schahn, R. (1996): Expression cloning of the Golgi CMP-sialic acid transporter. Proc. Natl. Acad. Sci. USA 93:7572-7576
Gerardy-Schahn, R., Bethe, A., Brennecke, T., Mühlenhoff, M., Eckhardt, M., Ziesing, S., Lottspeich, F., and Frosch, M. (1995): Molecular cloning and functional expression of bacteriophage PK1E encoded endoneuraminidase EndoNE. Mol. Microbiol. 16:441-450
Eckhardt, M., Mühlenhoff, M., Bethe, A., Koopman, J., Frosch, M., and Gerardy-Schahn, R. (1995): Molecular characterization of eukaryotic polysialyltransferase-1. Nature 373:715-718
Gerardy-Schahn, R., Eckhardt, M., Ledermann, J., and Kemshead, J.T. (1994): Topography of NCAM antigenic epitopes recognized by SCLC cluster-1 antibodies. A consensus view. Int. J. Cancer, Suppl. 8:27-29
Gerardy-Schahn, R., and Eckhardt, M. (1994): Hot spots of antigenicity in the neural cell adhesion molecule NCAM. Int. J. Cancer, Suppl. 8:38-42
Madry, N. (1994): Characterization of tumor-associated neural cell adhesion molecule in human serum. Cancer Res. 54:2598-2603
