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Polyglutamine Disorders : Advances in Experimental Medicine and Biology - Clevio Nobrega

Polyglutamine Disorders

Advances in Experimental Medicine and Biology

By: Clevio Nobrega (Editor), Luis Pereira de Almeida (Editor)

Paperback Published: 6th June 2019
ISBN: 9783319891033
Number Of Pages: 469

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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: 9783319891033
ISBN-10: 3319891030
Series: Advances in Experimental Medicine and Biology
Audience: General
Format: Paperback
Language: English
Number Of Pages: 469
Published: 6th June 2019
Publisher: SPRINGER VERLAG GMBH
Country of Publication: CH
Dimensions (cm): 23.39 x 15.6  x 2.46
Weight (kg): 0.67

Earn 575 Qantas Points
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