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Description

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.
Pages
469 pages
Collection
n.c
Parution
2018-02-09
Marque
Springer
EAN papier
9783319717784
EAN PDF
9783319717791
Informations sur l'ebook
Nombre pages copiables
4
Nombre pages imprimables
46
Taille du fichier
9892 Ko
Ce livre est accessible aux handicaps Voir les informations sur l'accessibilité
Prix
126,59 €
EAN EPUB
9783319717791
Informations sur l'ebook
Nombre pages copiables
4
Nombre pages imprimables
46
Taille du fichier
4311 Ko
Ce livre est accessible aux handicaps Voir les informations sur l'accessibilité
Prix
126,59 €
Clevio Nobrega Ph.D. and Luis Pereira de Almeida Ph.D.
University of Coimbra, Center for Neurosciences and Cell Biology, FMUC, Coimbra, Portugal

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