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Advances in Experimental Medicine and Biology

Polyglutamine Disorders

Editors: Nóbrega, Clévio, Pereira de Almeida, Luís (Eds.)

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  • Provides a cutting-edge review of polyglutamine diseases
  • Includes chapters that explore each disorder in depth, as well as gene and cell therapies
  • Shows the involvement of leading global researchers in the field of polyglutamine disorders 
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eBook $169.00
price for USA in USD (gross)
  • ISBN 978-3-319-71779-1
  • Digitally watermarked, DRM-free
  • Included format: PDF, EPUB
  • ebooks can be used on all reading devices
  • Immediate eBook download after purchase
Hardcover $219.99
price for USA in USD
  • ISBN 978-3-319-71778-4
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
Softcover $219.99
price for USA in USD
  • ISBN 978-3-319-89103-3
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
About this book

This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies’ development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. 

Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington’s disease (HD); spinal–bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17).

The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.


About the authors

Clevio Nobrega Ph.D. and Luis Pereira de Almeida Ph.D.University of Coimbra, Center for Neurosciences and Cell Biology, FMUC, Coimbra, Portugal

Table of contents (22 chapters)

Table of contents (22 chapters)
  • Clinical Features of Huntington’s Disease

    Pages 1-28

    Ghosh, Rhia (et al.)

  • Genetic Rodent Models of Huntington Disease

    Pages 29-57

    Stricker-Shaver, J. (et al.)

  • Mitochondrial Dysfunction in Huntington’s Disease

    Pages 59-83

    Carmo, Catarina (et al.)

  • RNA Related Pathology in Huntington’s Disease

    Pages 85-101

    Neueder, Andreas (et al.)

  • X-Linked Spinal and Bulbar Muscular Atrophy: From Clinical Genetic Features and Molecular Pathology to Mechanisms Underlying Disease Toxicity

    Pages 103-133

    Cortes, Constanza J. (et al.)

Buy this book

eBook $169.00
price for USA in USD (gross)
  • ISBN 978-3-319-71779-1
  • Digitally watermarked, DRM-free
  • Included format: PDF, EPUB
  • ebooks can be used on all reading devices
  • Immediate eBook download after purchase
Hardcover $219.99
price for USA in USD
  • ISBN 978-3-319-71778-4
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
Softcover $219.99
price for USA in USD
  • ISBN 978-3-319-89103-3
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
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Bibliographic Information

Bibliographic Information
Book Title
Polyglutamine Disorders
Editors
  • Clévio Nóbrega
  • Luís Pereira de Almeida
Series Title
Advances in Experimental Medicine and Biology
Series Volume
1049
Copyright
2018
Publisher
Springer International Publishing
Copyright Holder
Springer International Publishing AG
eBook ISBN
978-3-319-71779-1
DOI
10.1007/978-3-319-71779-1
Hardcover ISBN
978-3-319-71778-4
Softcover ISBN
978-3-319-89103-3
Series ISSN
0065-2598
Edition Number
1
Number of Pages
VIII, 469
Number of Illustrations
2 b/w illustrations, 39 illustrations in colour
Topics