Matthias Eckhardt

Summary

Affiliation: University of Bonn
Country: Germany

Publications

  1. pmc N-glycosylation is required for full enzymic activity of the murine galactosylceramide sulphotransferase
    Matthias Eckhardt
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Nussallee 11, D 53115 Bonn, Germany
    Biochem J 368:317-24. 2002
  2. ncbi request reprint The role and metabolism of sulfatide in the nervous system
    Matthias Eckhardt
    Institute of Physiological Chemistry, University of Bonn, Nussallee 11, 53115 Bonn, Germany
    Mol Neurobiol 37:93-103. 2008
  3. ncbi request reprint Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy
    Matthias Eckhardt
    Institute of Physiological Chemistry, University of Bonn, 53115 Bonn, Germany
    J Neurosci 27:9009-21. 2007
  4. pmc A mammalian fatty acid hydroxylase responsible for the formation of alpha-hydroxylated galactosylceramide in myelin
    Matthias Eckhardt
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Nussallee 11, 53115 Bonn, Germany
    Biochem J 388:245-54. 2005
  5. ncbi request reprint Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy
    Hariharasubramanian Ramakrishnan
    Institute of Physiological Chemistry, Rheinische Friedrich Wilhelms University of Bonn, 53115 Bonn, Germany
    J Neurosci 27:9482-90. 2007
  6. doi request reprint Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration
    Inge Zöller
    Institute of Physiological Chemistry, University of Bonn, 53115 Bonn, Germany
    J Neurosci 28:9741-54. 2008
  7. ncbi request reprint Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase
    Simon N Fewou
    Institut fur Physiologische Chemie, University of Bonn, Germany
    J Neurochem 94:469-81. 2005
  8. pmc Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes
    Christina Ginkel
    Molecular Genetics, University of Bonn, 53115 Bonn, Germany
    J Biol Chem 287:41888-902. 2012
  9. pmc Molecular characterization of N-acetylaspartylglutamate synthetase
    Ivonne Becker
    Institute of Biochemistry and Molecular Biology, University of Bonn, D 53115 Bonn, Germany
    J Biol Chem 285:29156-64. 2010
  10. pmc Myelination in the absence of UDP-galactose:ceramide galactosyl-transferase and fatty acid 2 -hydroxylase
    Marion Meixner
    Institute of Biochemistry and Molecular Biology, University of Bonn, Germany
    BMC Neurosci 12:22. 2011

Collaborators

Detail Information

Publications26

  1. pmc N-glycosylation is required for full enzymic activity of the murine galactosylceramide sulphotransferase
    Matthias Eckhardt
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Nussallee 11, D 53115 Bonn, Germany
    Biochem J 368:317-24. 2002
    ..Expression of fully active CST in a CHO-glycosylation mutant lacking N-acetylglucosaminyltransferase I demonstrated that condensation of the N-linked pentamannosyl-core structure is sufficient to form a fully active enzyme...
  2. ncbi request reprint The role and metabolism of sulfatide in the nervous system
    Matthias Eckhardt
    Institute of Physiological Chemistry, University of Bonn, Nussallee 11, 53115 Bonn, Germany
    Mol Neurobiol 37:93-103. 2008
    ..This review summarizes recent studies on the physiological and pathophysiological role of sulfatide using transgenic mice deficient in its synthesis or degradation...
  3. ncbi request reprint Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy
    Matthias Eckhardt
    Institute of Physiological Chemistry, University of Bonn, 53115 Bonn, Germany
    J Neurosci 27:9009-21. 2007
    ..These observations suggest that SGalCer accumulation in neurons contributes to disease phenotype...
  4. pmc A mammalian fatty acid hydroxylase responsible for the formation of alpha-hydroxylated galactosylceramide in myelin
    Matthias Eckhardt
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Nussallee 11, 53115 Bonn, Germany
    Biochem J 388:245-54. 2005
    ..Its further characterization will provide insight into the functional role of alpha-hydroxylation modification in myelin, skin and other organs...
  5. ncbi request reprint Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy
    Hariharasubramanian Ramakrishnan
    Institute of Physiological Chemistry, Rheinische Friedrich Wilhelms University of Bonn, 53115 Bonn, Germany
    J Neurosci 27:9482-90. 2007
    ..The approach described here may also be applicable to improve other mouse models of lysosomal as well as nonlysosomal disorders...
  6. doi request reprint Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration
    Inge Zöller
    Institute of Physiological Chemistry, University of Bonn, 53115 Bonn, Germany
    J Neurosci 28:9741-54. 2008
    ..Because axon degeneration appear to start rather early with respect to myelin degenerations, these lipids might be required for glial support of axon function...
  7. ncbi request reprint Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase
    Simon N Fewou
    Institut fur Physiologische Chemie, University of Bonn, Germany
    J Neurochem 94:469-81. 2005
    ..These mice developed a progressive hindlimb paralysis and demyelination in the CNS, demonstrating that tight control of UDP-galactose:ceramide galactosyltransferase expression is essential for myelin maintenance...
  8. pmc Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes
    Christina Ginkel
    Molecular Genetics, University of Bonn, 53115 Bonn, Germany
    J Biol Chem 287:41888-902. 2012
    ..Our results reveal an essential function of CerS1-derived ceramide in the regulation of cerebellar development and neurodevelopmentally regulated behavior...
  9. pmc Molecular characterization of N-acetylaspartylglutamate synthetase
    Ivonne Becker
    Institute of Biochemistry and Molecular Biology, University of Bonn, D 53115 Bonn, Germany
    J Biol Chem 285:29156-64. 2010
    ..Taken together our results strongly suggest that the identified gene encodes a NAAG synthetase. Its identification will enable further studies to examine the role of this abundant neuropeptide in the vertebrate nervous system...
  10. pmc Myelination in the absence of UDP-galactose:ceramide galactosyl-transferase and fatty acid 2 -hydroxylase
    Marion Meixner
    Institute of Biochemistry and Molecular Biology, University of Bonn, Germany
    BMC Neurosci 12:22. 2011
    ..In order to test this hypothesis, we have generated Cgt-/- mice with an additional deletion of the fatty acid 2-hydroxylase (Fa2h) gene...
  11. pmc N-acetylaspartylglutamate synthetase II synthesizes N-acetylaspartylglutamylglutamate
    Julia Lodder-Gadaczek
    Institute of Biochemistry and Molecular Biology, University of Bonn, D 53115 Bonn, Germany
    J Biol Chem 286:16693-706. 2011
    ..To our knowledge the presence of NAAG(2) in the vertebrate nervous system has not been described before. The physiological role of NAAG(2), e.g. whether it acts as a neurotransmitter, remains to be determined...
  12. pmc Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas
    Silke Imgrund
    Institute of Genetics, Division of Molecular Genetics, University of Bonn, Romerstrasse 164, 53117 Bonn, Germany
    J Biol Chem 284:33549-60. 2009
    ..Our results indicate that CERS2 activity supports different biological functions: maintenance of myelin, stabilization of the cerebellar as well as renal histological architecture, and protection against hepatocarcinomas...
  13. ncbi request reprint Oligodendrocyte-specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT-deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level
    Inge Zöller
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat, Bonn, Germany
    Glia 52:190-8. 2005
    ..Our results indicate that loss of CGT in oligodendrocytes is exclusively responsible for the myelin structural deficits, demyelination, and behavioral abnormalities in CGT-deficient mice...
  14. ncbi request reprint Ceramide synthase 4 deficiency in mice causes lipid alterations in sebum and results in alopecia
    Philipp Ebel
    Molecular Genetics, Life and Medical Sciences Institute LIMES, University of Bonn, 53115 Bonn, Germany
    Biochem J 461:147-58. 2014
    ..Cers4-/- mice at 12 months old display additional epidermal tissue destruction due to dilated and obstructed pilary canals. Mass spectrometric analyses additionally show a strong decrease in C20-containing sphingolipids. ..
  15. ncbi request reprint Cerebroside sulfotransferase forms homodimers in living cells
    Afshin Yaghootfam
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Germany
    Biochemistry 46:9260-9. 2007
    ..In contrast to full-length CST, a fusion protein of the amino-terminal 36 amino acids of CST fused to EGFP was exclusively found as a monomer but nevertheless showed Golgi localization...
  16. ncbi request reprint Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2
    Ivonne Becker
    Institute of Physiological Chemistry, University of Bonn, 53115 Bonn, Germany
    Histochem Cell Biol 129:233-41. 2008
    ..Furthermore, CerS2 was the predominant CerS in Schwann cells of sciatic nerves. These data suggest that CerS2 is important for the synthesis of dihydroceramide used for synthesis of myelin sphingolipids...
  17. ncbi request reprint Elevated sulfatide levels in neurons cause lethal audiogenic seizures in mice
    Rebekka van Zyl
    Institute of Biochemistry and Molecular Biology, University of Bonn, Bonn, Germany
    J Neurochem 112:282-95. 2010
    ..CGT-transgenic mice will be a useful model to further investigate how sulfatide affects functional properties of neurons...
  18. ncbi request reprint Down-regulation of polysialic acid is required for efficient myelin formation
    Simon Ngamli Fewou
    Institute of Physiological Chemistry and Institute of Anatomy, University of Bonn, D 53115 Bonn, Germany
    J Biol Chem 282:16700-11. 2007
    ..Furthermore, myelin of transgenic mice exhibited structural abnormalities like redundant myelin and axonal degeneration, indicating that the down-regulation of PSA is also necessary for myelin maintenance...
  19. pmc Inactivation of ceramide synthase 6 in mice results in an altered sphingolipid metabolism and behavioral abnormalities
    Philipp Ebel
    Molecular Genetics, Life and Medical Sciences Institute LIMES, University of Bonn, 53115 Bonn, Germany
    J Biol Chem 288:21433-47. 2013
    ..Using newly developed antibodies that specifically recognize the CerS6 protein we show that the endogenous CerS6 protein is N-glycosylated and expressed in several tissues of mice, mainly kidney, small and large intestine, and brain. ..
  20. ncbi request reprint Reduced brain cholesterol content in arylsulfatase A-deficient mice
    Dieter Lutjohann
    Institute of Clinical Pharmacology, University of Bonn, Germany
    Biochem Biophys Res Commun 344:647-50. 2006
    ..Since high cholesterol levels are important for myelination, and various cellular processes, like vesicular trafficking and signal transduction, reduced cholesterol levels might be an important factor in the molecular pathology of MLD...
  21. ncbi request reprint Delay of myelin formation in arylsulphatase A-deficient mice
    Afshin Yaghootfam
    Institut fur Physiologische Chemie, Rheinische Friedrich Wilhelms Universitat Bonn, Nussallee 11, 53115 Bonn, Germany
    Eur J Neurosci 21:711-20. 2005
    ..Our data demonstrate a delay in myelin formation in ASA-/- mice. This raises the possibility that similar alterations in MLD patients may contribute to the pathology of the disease...
  22. doi request reprint Vesicular uptake of N-acetylaspartylglutamate is catalysed by sialin (SLC17A5)
    Julia Lodder-Gadaczek
    Institute of Biochemistry and Molecular Biology, University of Bonn, Nussallee 11, D 53115 Bonn, Germany
    Biochem J 454:31-8. 2013
    ..We also show that sialin is most probably the major and possibly only vesicular transporter for NAAG and NAAG2, because ATP-dependent transport of both peptides was not detectable in vesicles isolated from sialin-deficient mice. ..
  23. ncbi request reprint Pathology and current treatment of neurodegenerative sphingolipidoses
    Matthias Eckhardt
    Institute of Biochemistry and Molecular Biology, University of Bonn, Nussallee 11, 53115 Bonn, Germany
    Neuromolecular Med 12:362-82. 2010
    ..In the future, neural stem cell therapy and gene therapy may become an option for these disorders...
  24. doi request reprint Early signs of neurolipidosis-related behavioural alterations in a murine model of metachromatic leukodystrophy
    Stijn Stroobants
    Laboratory of Biological Psychology, Department of Psychology, University of Leuven, Leuven, Belgium
    Behav Brain Res 189:306-16. 2008
    ..Six-month-old ASA(-/-) mice also showed decreased response rates in scheduled operant responding. The present findings could provide relevant behavioural outcome measures for further use of this murine MLD model in preclinical studies...
  25. ncbi request reprint Sulfatide binding properties of murine and human antiganglioside antibodies
    Kate Townson
    Division of Clinical Neurosciences, Glasgow Biomedical Research Centre, University of Glasgow, G12 8TA Scotland
    Glycobiology 17:1156-66. 2007
    ..These data demonstrate that sulfatide binding is a common property of antiganglioside antibodies that may provide functional insights into, and consequences for this component of the innate immune repertoire...
  26. doi request reprint 3,5-Dihydro-imidazo[4,5-d]pyridazin-4-ones: a class of potent DPP-4 inhibitors
    Matthias Eckhardt
    Department of Chemical Research, Boehringer Ingelheim Pharma GmbH and Co KG, 88400 Biberach, Germany
    Bioorg Med Chem Lett 18:3158-62. 2008
    ....