Polyglutamine Disorders

Szerző: Clévio Nóbrega, Luís Pereira de Almeida

Nyelv:

Kötés: Kemény kötésű

Kiadó: Springer International Publishing AG

Elérhetőség: Beszállítói készleten alacsony példányszámban

Küldés 11-15 napon belül

71 933 Ft

This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two mai...

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Szerző

Clévio Nóbrega, Luís Pereira de Almeida

Nyelv

Angol

Kötés

Könyv - Kemény kötésű

Kiadva

2018

oldal

469

EAN

9783319717784

ISBN

3319717782

Enbook ID

18368703

Kiadó

Springer International Publishing AG

Súly

8395

Méretek

155 x 235 x 32

Teljes leírás

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.

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