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Peroxisomal Disorders and Regulation of Genes

  • Frank Roels
  • Myriam Baes
  • Sylvia De Bie

Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 544)

Table of contents

  1. Front Matter
    Pages i-xiv
  2. Hanna Mandel, Stanley H. Korman
    Pages 9-30
  3. Jukka KallijÄrvi, Anna-Elina Lehesjoki, Marita Lipsanen-Nyman
    Pages 31-37
  4. Frank Roels, Marianne Depreter, Marc Espeel, Katharina D’herde, Ingrid Kerckaert, Joseph Vamecq et al.
    Pages 39-54
  5. Gregory Chevillard, Marie-Claude ClÉmencet, Philippe Etienne, Pascal Martin, Thierry Pineau, Norbert Latruffe et al.
    Pages 55-56
  6. Bart P Leroy, Chris R Hogg, Pamela R Rath, Vicky Mcbain, Philippe Kestelyn, Alan C Bird et al.
    Pages 57-58
  7. Jeannette Gootjes, Petra A. W. Mooijer, Conny Dekker, Peter G. Barth, Bwee Tien Poll-The, Hans R. Waterham et al.
    Pages 67-68
  8. Daan M. Van Den Brink, Pedro Brites, Janet Haasjes, Anthony S. Wierzbicki, John Mitchell, Michelle Lambert-Hamill et al.
    Pages 69-70
  9. Ann K. Heinzer, Martina C. Mcguinness, Jyh-Feng Lu, O. Colin Stine, Heming Wei, Mark Van Der Vlies et al.
    Pages 75-93
  10. Andreas Holzinger, Peter U. Mayerhofer, Esther M. Maier, Adelbert A. Roscher, Johannes Berger
    Pages 95-96
  11. Frank Roels, Jean-Marie Saudubray, Marisa Giros, Hanna Mandel, FranÇois Eyskens, Nieves Saracibar et al.
    Pages 97-106
  12. Jeannette Gootjes, Hanna Mandel, Petra A.W. Mooijer, Frank Roels, Hans R. Waterham, Ronald J.A. Wanders
    Pages 107-111
  13. Osman El-Maarri
    Pages 135-144
  14. Joanna B. Grabarek
    Pages 145-158
  15. Martine De Rycke
    Pages 159-168
  16. Wim Vanden Berghe, Linda Vermeulen, Philippe Delerive, Karolien De Bosscher, Bart Staels, Guy Haegeman
    Pages 181-196
  17. Osman El-Maarri
    Pages 197-204
  18. Joanna B. Grabarek, Magdalena Zernicka-Goetz
    Pages 205-216
  19. Alexandra M. Gouveia, Carla P. GuimÂres, MÁrcia E. Oliveira, Carlos Reguenga, Clara SÁ-Miranda, Jorge E. Azevedo
    Pages 219-220
  20. Ania C. Muntau, Adelbert A. Roscher, Wolf-H Kunau, Gabriele Dodt
    Pages 221-224
  21. Norbert Latruffe, Valerie Nicolas-FrancÈs, Marie-Claude Clemencet, Franck Hansmannel, Gregory Chevillard, Philippe Etienne et al.
    Pages 225-236
  22. Marianne Depreter, Jo Vandesompele, Sacha Ferdinandusse, Frank Speleman, Frank Roels
    Pages 237-242
  23. Fabien Gueugnon, Fabrice Lambert, Catherine Gondcaille, Stephane Fourcade, Jerome Bellenger, Francoise Cadepond et al.
    Pages 245-245
  24. Anna W.M. Zomer, Paul T. Van Der Saag, Bwee Tien Poll-The
    Pages 247-254
  25. Marianne Depreter, Tracy Walker, Sonja Beken, Karen De Smet, Peggy Papeleu, Tom Tytgat et al.
    Pages 255-264
  26. Marisa Giros, Montse Ruiz
    Pages 265-270
  27. Annamaria Cimini, Loredana Cristiano, Antonietta Bernardo, Elisabetta Benedetti, Silvia Di Loreto, Maria Paola Cerù
    Pages 271-280
  28. Maurice Bugaut, Stephane Fourcade, Catherine Gondcaille, Fabien Gueugnon, Marianne Depreter, Frank Roels et al.
    Pages 281-291
  29. Ronald J.A. Wanders, Carlo W.T. Van Roermund, Wouter F. Visser, Sacha Ferdinandusse, Gerbert A. Jansen, Daan M. Van den Brink et al.
    Pages 293-302
  30. Matthew D. Lloyd, Mridul Mukherji, Nadia J. Kershaw, Winnie Chien, Anthony S. Wierzbicki, Christopher J. Schofield
    Pages 303-304
  31. Veerle Foulon, Minne Casteels, Guy P. Mannaerts, Bruce D. Gelb, Paul P. VanVeldhoven
    Pages 305-306
  32. Christiane Van Den Branden, Joseph Vamecq
    Pages 307-314
  33. Werner J. Kovacs, Skaidrite Krisans
    Pages 315-327
  34. Sietske Hogenboom, Gerrit Jan Romeijn, Sander M. Houten, Myriam Baes, Ronald J.A. Wanders, Hans R. Waterham
    Pages 329-330
  35. Isabelle Weinhofer, Sonja Forss-Petter, Mihaela Zigman, Johannes Berger
    Pages 331-332
  36. Catherine Le Jossic-Corcos, Isabelle Zaghini, Celine Gonthier, Emmanuelle Logette, Paulette Bournot
    Pages 333-334
  37. Akhlaq A. Farooqui, Wei-Yi Ong, Lloyd A. Horrocks
    Pages 335-354
  38. Claus Rodemer, Thanh-Phuong Thai, Britta Brügger, Karin Gorgas, Wilhelm Just
    Pages 355-368
  39. Hugo W. Moser, Gerald V. Raymond, Wolfgang Koehler, Piotr Sokolowski, Folker Hanefeld, Georg Christoph Korenke et al.
    Pages 369-387
  40. Back Matter
    Pages 389-429

About these proceedings

Introduction

In most peroxisomal disorders the nervous system is severely affected which explains the clinical and community burden they represent. This is the first book to focus not only on the mutations causing these inherited illnesses, but also on mechanisms that regulate, suppress or enhance expression of genes and their products (enzymes). Indeed since the success and completion of the Human Genome Project all genes (coding DNA sequences) are known. However, of many, their function, and the role of the gene product has not been determined. An example is X-linked adrenoleukodystrophy, the most frequent peroxisomal disorder. Children are born healthy, but in more than 1 out of 3, demyelination of the brain starts unpredictably and they die in a vegetative state. The gene mutated in most families has been known for 10 years; but the true role of the encoded protein, ALDp, is still speculative; and within the same family, very severe and asymptomatic clinical histories co-exist, unexplained by the mutation.

Keywords

DNA Lipid Nervous System Oxidation RNA biosynthesis enzymes gene expression genes metabolism molecular mechanisms

Editors and affiliations

  • Frank Roels
    • 1
  • Myriam Baes
    • 2
  • Sylvia De Bie
    • 1
  1. 1.Ghent UniversityGhentBelgium
  2. 2.K.U. LeuvenLeuvenBelgium

Bibliographic information

  • DOI https://doi.org/10.1007/978-1-4419-9072-3
  • Copyright Information Springer Science+Business Media New York 2003
  • Publisher Name Springer, Boston, MA
  • eBook Packages Springer Book Archive
  • Print ISBN 978-1-4613-4782-8
  • Online ISBN 978-1-4419-9072-3
  • Series Print ISSN 0065-2598
  • Buy this book on publisher's site
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