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

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
  • About this book