Advances in Experimental Medicine and Biology

Peroxisomal Disorders and Regulation of Genes

Editors: Roels, Frank, Baes, Myriam, Delanghe, R. (Eds.)

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About this book

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.

Table of contents (47 chapters)

  • Why Study Regulation of Genes in Inherited Disorders?

    Roels, Frank

    Pages 1-8

  • Phenotypic Variability (Heterogeneity) of Peroxisomal Disorders

    Mandel, Hanna (et al.)

    Pages 9-30

  • Mulibrey Nanism - a Novel Peroxisomal Disorder

    KallijÄrvi, Jukka (et al.)

    Pages 31-37

  • Peroxisomes during Development and in Distinct Cell Types

    Roels, Frank (et al.)

    Pages 39-54

  • Tissue-specific Expression of Two Peroxisomal 3-ketoacyl-CoA Thiolase Genes in Wild and PPARα-null Mice and Induction by Fenofibrate

    Chevillard, Gregory (et al.)

    Pages 55-56

Buy this book

eBook $199.00
price for USA (gross)
  • ISBN 978-1-4419-9072-3
  • Digitally watermarked, DRM-free
  • Included format: PDF
  • ebooks can be used on all reading devices
  • Immediate eBook download after purchase
Hardcover $349.00
price for USA
  • ISBN 978-0-306-48174-1
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
Softcover $259.00
price for USA
  • ISBN 978-1-4613-4782-8
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
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Bibliographic Information

Bibliographic Information
Book Title
Peroxisomal Disorders and Regulation of Genes
Editors
  • Frank Roels
  • Myriam Baes
  • R. Delanghe
Series Title
Advances in Experimental Medicine and Biology
Series Volume
544
Copyright
2003
Publisher
Springer US
Copyright Holder
Springer Science+Business Media New York
eBook ISBN
978-1-4419-9072-3
DOI
10.1007/978-1-4419-9072-3
Hardcover ISBN
978-0-306-48174-1
Softcover ISBN
978-1-4613-4782-8
Series ISSN
0065-2598
Edition Number
1
Number of Pages
XIV, 429
Topics