Wilhelm Stoffel

Summary

Affiliation: University of Cologne
Country: Germany

Publications

  1. ncbi Delta6-desaturase (FADS2) deficiency unveils the role of omega3- and omega6-polyunsaturated fatty acids
    Wilhelm Stoffel
    Center of Molecular Medicine CMMC, Laboratory of Molecular Neurosciences, Institute of Biochemistry, University of Cologne, Cologne, Germany
    EMBO J 27:2281-92. 2008
  2. ncbi Functional analysis of acid and neutral sphingomyelinases in vitro and in vivo
    W Stoffel
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, University of Cologne, Koln, Germany
    Chem Phys Lipids 102:107-21. 1999
  3. ncbi Myelin glycolipids and their functions
    W Stoffel
    Molecular Neuroscience Laboratory, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Germany
    Curr Opin Neurobiol 7:654-61. 1997
  4. ncbi Functional analysis of glutamate transporters in excitatory synaptic transmission of GLAST1 and GLAST1/EAAC1 deficient mice
    Wilhelm Stoffel
    Laboratory of Molecular Neuroscience, Faculty of Medicine, Center of Molecular Medicine Cologne, Institute of Biochemistry, University of Cologne, Joseph Stelzmannstrasse 52, D 50931 Köln, Germany
    Brain Res Mol Brain Res 128:170-81. 2004
  5. ncbi Neutral sphingomyelinase 2 (smpd3) in the control of postnatal growth and development
    Wilhelm Stoffel
    Laboratory of Molecular Neurosciences, Center of Molecular Medicine, Center of Biochemistry, Faculty of Medicine, University of Cologne, D 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 102:4554-9. 2005
  6. ncbi Neutral sphingomyelinase (SMPD3) deficiency causes a novel form of chondrodysplasia and dwarfism that is rescued by Col2A1-driven smpd3 transgene expression
    Wilhelm Stoffel
    University of Cologne, Laboratory of Molecular Neurosciences, Joseph Stelzmannstrasse 52, Cologne, Germany 50931, USA
    Am J Pathol 171:153-61. 2007
  7. ncbi Obesity resistance of the stearoyl-CoA desaturase-deficient (scd1-/-) mouse results from disruption of the epidermal lipid barrier and adaptive thermoregulation
    Erika Binczek
    Laboratory of Molecular Neurosciences, Center of Molecular Medicine CMMC, Center of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmann Str 52, D 50931 Köln, Germany
    Biol Chem 388:405-18. 2007
  8. ncbi Neutral sphingomyelinase 1 deficiency in the mouse causes no lipid storage disease
    Markus Zumbansen
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Cologne, Germany
    Mol Cell Biol 22:3633-8. 2002
  9. ncbi Early onset of axonal degeneration in double (plp-/-mag-/-) and hypomyelinosis in triple (plp-/-mbp-/-mag-/-) mutant mice
    T Uschkureit
    Laboratory for Molecular Neuroscience, University of Cologne, D 50931 Cologne, Germany, and Institute of Anatomy, University of Bonn, D 53115 Bonn, Germany
    J Neurosci 20:5225-33. 2000
  10. ncbi Nerve conduction velocity measurements reveal the functional deficit in ceramide galactosyl transferase-deficient (cgt-/-) mice
    Walter F Haupt
    Department of Neurology, University of Cologne, Joseph Stelzmann Str 9 D 50924 Cologne, Germany
    J Neurol Sci 217:83-8. 2004

Collaborators

Detail Information

Publications33

  1. ncbi Delta6-desaturase (FADS2) deficiency unveils the role of omega3- and omega6-polyunsaturated fatty acids
    Wilhelm Stoffel
    Center of Molecular Medicine CMMC, Laboratory of Molecular Neurosciences, Institute of Biochemistry, University of Cologne, Cologne, Germany
    EMBO J 27:2281-92. 2008
    ..The fads2 -/- mouse is an auxotrophic mutant. It is anticipated that FADS2 will become a major focus in membrane, haemostasis, inflammation and atherosclerosis research...
  2. ncbi Functional analysis of acid and neutral sphingomyelinases in vitro and in vivo
    W Stoffel
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, University of Cologne, Koln, Germany
    Chem Phys Lipids 102:107-21. 1999
    ....
  3. ncbi Myelin glycolipids and their functions
    W Stoffel
    Molecular Neuroscience Laboratory, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Germany
    Curr Opin Neurobiol 7:654-61. 1997
    ..Additional knock-out studies should provide important insights into the various functions of glycolipids in myelinogenesis and myelin structure...
  4. ncbi Functional analysis of glutamate transporters in excitatory synaptic transmission of GLAST1 and GLAST1/EAAC1 deficient mice
    Wilhelm Stoffel
    Laboratory of Molecular Neuroscience, Faculty of Medicine, Center of Molecular Medicine Cologne, Institute of Biochemistry, University of Cologne, Joseph Stelzmannstrasse 52, D 50931 Köln, Germany
    Brain Res Mol Brain Res 128:170-81. 2004
    ....
  5. ncbi Neutral sphingomyelinase 2 (smpd3) in the control of postnatal growth and development
    Wilhelm Stoffel
    Laboratory of Molecular Neurosciences, Center of Molecular Medicine, Center of Biochemistry, Faculty of Medicine, University of Cologne, D 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 102:4554-9. 2005
    ..The smpd3(-/-) mouse might mimic a form of human combined pituitary hormone deficiency...
  6. ncbi Neutral sphingomyelinase (SMPD3) deficiency causes a novel form of chondrodysplasia and dwarfism that is rescued by Col2A1-driven smpd3 transgene expression
    Wilhelm Stoffel
    University of Cologne, Laboratory of Molecular Neurosciences, Joseph Stelzmannstrasse 52, Cologne, Germany 50931, USA
    Am J Pathol 171:153-61. 2007
    ..The rescue of smpd3 in vivo has implications for future research into dwarfism and, particularly, growth and development of the skeletal system and for current screening and future treatment of combined dwarfism and chondrodysplasia...
  7. ncbi Obesity resistance of the stearoyl-CoA desaturase-deficient (scd1-/-) mouse results from disruption of the epidermal lipid barrier and adaptive thermoregulation
    Erika Binczek
    Laboratory of Molecular Neurosciences, Center of Molecular Medicine CMMC, Center of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmann Str 52, D 50931 Köln, Germany
    Biol Chem 388:405-18. 2007
    ..Finally, the occurrence of SCD1 and a newly described hSCD5 (ACOD4) gene in humans suggests that the scd1-/- mouse mutant might be a valuable animal model for the study of human skin diseases associated with epidermal barrier defects...
  8. ncbi Neutral sphingomyelinase 1 deficiency in the mouse causes no lipid storage disease
    Markus Zumbansen
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Cologne, Germany
    Mol Cell Biol 22:3633-8. 2002
    ..The unaltered metabolism of lysophosphatidylcholine or lyso-platelet-activating factor excludes the proposed role of nSMase 1 as a lysophospholipase C...
  9. ncbi Early onset of axonal degeneration in double (plp-/-mag-/-) and hypomyelinosis in triple (plp-/-mbp-/-mag-/-) mutant mice
    T Uschkureit
    Laboratory for Molecular Neuroscience, University of Cologne, D 50931 Cologne, Germany, and Institute of Anatomy, University of Bonn, D 53115 Bonn, Germany
    J Neurosci 20:5225-33. 2000
    ..The longevity of these dysmyelinosis mouse mutants recommends them as suitable models for the long-term evaluation of stem cell therapeutic strategies...
  10. ncbi Nerve conduction velocity measurements reveal the functional deficit in ceramide galactosyl transferase-deficient (cgt-/-) mice
    Walter F Haupt
    Department of Neurology, University of Cologne, Joseph Stelzmann Str 9 D 50924 Cologne, Germany
    J Neurol Sci 217:83-8. 2004
    ..These electrophysiologic data provide additional functional parameters to the neuropathology of a new form of a dysmyelinosis...
  11. ncbi Disruption of mitochondrial beta -oxidation of unsaturated fatty acids in the 3,2-trans-enoyl-CoA isomerase-deficient mouse
    Uwe Janssen
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmannstrasse 52, D 50931 Cologne, Germany
    J Biol Chem 277:19579-84. 2002
    ....
  12. ncbi Rumpshaker-like proteolipid protein (PLP) ratio in a mouse model with unperturbed structural and functional integrity of the myelin sheath and axons in the central nervous system
    T Uschkureit
    Laboratory for Molecular Neuroscience, University of Cologne, Cologne, Germany
    Glia 35:63-71. 2001
    ....
  13. ncbi Functional analysis in vivo of the double mutant mouse deficient in both proteolipid protein (PLP) and myelin basic protein (MBP) in the central nervous system
    W Stoffel
    Institute of Biochemistry, D 50931 Köln, Germany
    Cell Tissue Res 289:195-206. 1997
    ..Neuromotor activity and coordination are considerably improved compared to the shiverer trait...
  14. ncbi Ceramide UDPgalactosyltransferase from myelinating rat brain: purification, cloning, and expression
    S Schulte
    Institute of Biochemistry, Medical Faculty of the University of Cologne, Germany
    Proc Natl Acad Sci U S A 90:10265-9. 1993
    ..The amino acid sequence of CGT shows significant homology to mammalian UDPglucuronyltransferases, which suggests a common evolutionary origin of these enzymes...
  15. ncbi Functional analysis of the glycosylation of murine acid sphingomyelinase
    D Newrzella
    Institute of Biochemistry I, Faculty of Medicine, University of Cologne, Joseph Stelzmann Str 52, 50931 Cologne, Germany
    J Biol Chem 271:32089-95. 1996
    ..32PO4 labeling revealed that the glycosylation mutants of ASM were phosphorylated predominantly at mannose residues of oligosaccharides linked to N-84, N-333, and N-393...
  16. ncbi Galactosphingolipids and axono-glial interaction in myelin of the central nervous system
    A Bosio
    Molecular Neuroscience Laboratory, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmann Strasse 52, D 50931 Cologne, Germany
    Cell Tissue Res 292:199-210. 1998
    ..Increased intracellular concentrations of free ceramides do not trigger apoptosis of oligodendrocytes...
  17. ncbi Oligodendrocytes expressing exclusively the DM20 isoform of the proteolipid protein gene: myelination and development
    Olaf Spörkel
    Laboratory of Molecular Neuroscience, University of Cologne, Cologne, Germany
    Glia 37:19-30. 2002
    ....
  18. ncbi Molecular cloning and characterization of the mouse CGT gene encoding UDP-galactose ceramide-galactosyltransferase (cerebroside synthetase)
    A Bosio
    Institut fur Biochemie, Medizinische Fakultat, Universitat Koln, Joseph Stelzmann Strasse 52, Cologne, D 50931, Germany
    Genomics 35:223-6. 1996
    ..Putative transcription start sites were determined by primer extension experiments. The CGT gene locus is highly conserved during evolution...
  19. ncbi Light and electron microscopic analysis of the central and peripheral nervous systems of acid sphingomyelinase-deficient mice resulting from gene targeting
    T A Kuemmel
    Institute of Pathology, Medical Faculty, University of Cologne, Germany
    J Neuropathol Exp Neurol 56:171-9. 1997
    ..The morphological changes of the central and peripheral nervous systems in the homozygous aSmase-/- mouse line closely resemble those in human NPA...
  20. ncbi Cloned mammalian neutral sphingomyelinase: functions in sphingolipid signaling?
    S Tomiuk
    Laboratory of Molecular Neurosciences, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmann Strasse 52, D 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 95:3638-43. 1998
    ..Tumor necrosis factor alpha triggers the ERK1 pathway in none of the cell lines. The cloned nSMase will facilitate further controlled experiments aiming at the definition of a possible role of ceramide as signal transduction molecule...
  21. ncbi Pathology of visceral organs and bone marrow in an acid sphingomyelinase deficient knock-out mouse line, mimicking human Niemann-Pick disease type A. A light and electron microscopic study
    T A Kuemmel
    Institute of Pathology, Medical Faculty, University of Cologne, Germany
    Pathol Res Pract 193:663-71. 1997
    ..The obvious similarity to its human counterpart along with an easy availability makes this animal model a valuable tool for further studies of Niemann-Pick disease type A...
  22. ncbi Human and murine serine-palmitoyl-CoA transferase--cloning, expression and characterization of the key enzyme in sphingolipid synthesis
    B Weiss
    Laboratory of Molecular Neuroscience, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Germany
    Eur J Biochem 249:239-47. 1997
    ..Further studies will be required to unravel the function of the LCB1 gene in mammalian cells...
  23. ncbi Functional breakdown of the lipid bilayer of the myelin membrane in central and peripheral nervous system by disrupted galactocerebroside synthesis
    A Bosio
    Institute of Biochemistry I, Medical Faculty, University of Cologne, Germany
    Proc Natl Acad Sci U S A 93:13280-5. 1996
    ..GalC and sGalC are essential for the unperturbed lipid bilayer of the myelin membrane of CNS and PNS. The severe dysmyelinosis leads to death of the cgt-/- mouse at the end of the myelination period...
  24. ncbi Tolerability and safety of two new preservative-free tear film substitutes
    Sven Dinslage
    Department of Ophthalmology, University of Cologne, Germany
    Cornea 21:352-5. 2002
    ..The tolerability, safety, and visual comfort of two new tear film substitutes were studied in a phase I clinical study...
  25. ncbi Perturbation of membrane microdomains reduces mitogenic signaling and increases susceptibility to apoptosis after T cell receptor stimulation
    M Nix
    Laboratory of Molecular Neuroscience, Institute of Biochemistry, Faculty of Medicine, University of Cologne, D 50931 Cologne, Germany
    Cell Death Differ 7:413-24. 2000
    ..Reduced MAPK activity-dependent FLICE-like inhibitory protein (FLIP) expression in asmase-/- T lymphocytes increases their sensitivity towards Fas-mediated apoptosis...
  26. ncbi Cotranslational integration of myelin proteolipid protein (PLP) into the membrane of endoplasmic reticulum: analysis of topology by glycosylation scanning and protease domain protection assay
    S Wahle
    Institute of Biochemistry, Faculty of Medicine, University of Cologne, Germany
    Glia 24:226-35. 1998
    ..These results are discussed with regard to the PLP biogenesis and the plasma membrane topology in PLP-expressing cells...
  27. ncbi Ceramide-independent CD28 and TCR signaling but reduced IL-2 secretion in T cells of acid sphingomyelinase-deficient mice
    B Stoffel
    Institute of Medical Microbiology and Hygiene, University of Cologne, Germany
    Eur J Immunol 28:874-80. 1998
    ..We conclude that ceramide generated by aSMase is not involved in CD28 signal transduction, but rather a perturbation of the secretory system is responsible for the impaired proliferation of aSMase-deficient splenocytes...
  28. ncbi Composition and biophysical properties of myelin lipid define the neurological defects in galactocerebroside- and sulfatide-deficient mice
    A Bosio
    Institute of Biochemistry, Faculty of Medicine, University of Cologne, Germany
    J Neurochem 70:308-15. 1998
    ..The loss of the two glycosphingolipid classes causes the breakdown of saltatory conductance of myelinated axons in the cgt-/- mouse...
  29. ncbi Tumor necrosis factor alpha activates NF-kappaB in acid sphingomyelinase-deficient mouse embryonic fibroblasts
    M Zumbansen
    Neuroscience Laboratory, Institute of Biochemistry, Faculty of Medicine, University of Cologne, Joseph Stelzmann Strasse 52, D 50931 Cologne, Germany
    J Biol Chem 272:10904-9. 1997
    ..TNF-alpha treatment induced the dissociation and degradation of IkappaB-alpha and the nuclear translocation of NF-kappaB in embryonic fibroblasts derived from asmase-/- and wild type mice indiscriminately...
  30. ncbi The human gene CGT encoding the UDP-galactose ceramide galactosyl transferase (cerebroside synthase): cloning, characterization, and assignment to human chromosome 4, band q26
    A Bosio
    Institut fur Biochemie, Medizinische Fakultat, Universitat Koln, Joseph Stelzmann Strasse 52, Cologne, D 50931, Germany
    Genomics 34:69-75. 1996
    ..The organization of the CGT gene and of the UGT (uridylglucuronosyltransferases) gene family suggests a correlation to functional domains of the encoded proteins...
  31. ncbi Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP
    T Weimbs
    Institute of Biochemistry, Medical Faculty, University of Cologne, Koeln, Germany
    Biochemistry 31:12289-96. 1992
    ..The structural data not only demand the revision of our concept of the membrane topology of PLP but will also promote more sophisticated studies on the mechanism of myelination and new functions of PLP...
  32. ncbi Human mitochondrial enoyl-CoA hydratase gene (ECHS1): structural organization and assignment to chromosome 10q26.2-q26.3
    U Janssen
    Medical Faculty, Universitat Koln, Joseph Stelzmann Strasse 52, Cologne, D 50931, Germany
    Genomics 40:470-5. 1997
    ..Alu repeat elements have been identified within the region -1052/-770 relative to the cap site and in intron 7. The human ECHS1 gene locus was assigned to chromosome 10q26.2-q26.3 by fluorescence in situ hybridization...
  33. ncbi UDP galactose:ceramide galactosyltransferase and glutamate/aspartate transporter. Copurification, separation and characterization of the two glycoproteins
    S Schulte
    Institute of Biochemistry, Medical Faculty, University of Cologne, Germany
    Eur J Biochem 233:947-53. 1995
    ....