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Polyglutamine disorders

Nobrega, Clevio.

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Polyglutamine disorders

Record Type:	Electronic resources : Monograph/item
Title/Author:	Polyglutamine disorders/ edited by Clevio Nobrega, Luis Pereira de Almeida.
other author:	Nobrega, Clevio.
Published:	Cham :Springer International Publishing : : 2018.,
Description:	viii, 469 p. :ill. (some col.), digital ;24 cm.
Contained By:	Springer eBooks
Subject:	Nervous system - Diseases -
Online resource:	http://dx.doi.org/10.1007/978-3-319-71779-1
ISBN:	9783319717791

Polyglutamine disorders
Polyglutamine disorders[electronic resource] /edited by Clevio Nobrega, Luis Pereira de Almeida. - Cham :Springer International Publishing :2018. - viii, 469 p. :ill. (some col.), digital ;24 cm. - Advances in experimental medicine and biology,v.10490065-2598 ;. - Advances in experimental medicine and biology ;v.1049..

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.

ISBN: 9783319717791

Standard No.: 10.1007/978-3-319-71779-1doiSubjects--Topical Terms:

682330
Nervous system
--Diseases

LC Class. No.: RC346.4

Dewey Class. No.: 616.8

Polyglutamine disorders
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245 0 0 $a Polyglutamine disorders $h [electronic resource] / $c edited by Clevio Nobrega, Luis Pereira de Almeida.
260 $a Cham : $b Springer International Publishing : $b Imprint: Springer, $c 2018.
300 $a viii, 469 p. : $b ill. (some col.), digital ; $c 24 cm.
490 1 $a Advances in experimental medicine and biology, $x 0065-2598 ; $v v.1049
520 $a 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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700 1 $a Nobrega, Clevio. $3 2114871
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