Special Issue "Molecular Mechanisms Underlying Huntington's Disease"


A special issue of Brain Sciences (ISSN 2076-3425).

Deadline for manuscript submissions: closed (31 March 2014)

Special Issue Editor

Guest Editor
Dr. Sybille Krauss
German Center for Neurodegenerative Diseases (DZNE), Biomedical Center (BMZ1) - Building 344, Sigmund-Freud-Str. 25, 53127 Bonn, Germany
Website: http://www.dzne.de/en/sites/bonn/research-groups/krauss.html
E-Mail: Sybille.Krauss@dzne.de

Special Issue Information

Dear Colleagues,

Huntington disease (HD) is a late-onset neurodegenerative disorder that manifests with severe movement disorder, dementia, and psychiatric disturbance. The disease is progressive and invariably fatal and to date there is no cure. HD is a monogenic, autosomal dominant disease. The prevalence for HD varies between different populations and specific HTT haplotypes have been associated with HD in distinct populations. HD is caused by an expansion of a CAG trinucleotide repeat in the huntingtin (HTT) gene that encodes a polyglutamine tract in the huntingtin protein (HTT). HD is the most common polyglutamine disorder and is widely accepted as a model disease for studying pathogenic mechanisms and developing therapies in these diseases. HTT is an essential protein that is expressed ubiquitously. However, HD-linked pathogenesis is observed only in the patient’s brains, where N-terminal cleavage fragments of the polyglutamine proteins accumulate into aggregates. The molecular and cellular pathways underlying neurodegeneration in HD are the focus of much research.

The current special issue is meant to collect a selected number of articles that show recent findings in molecular genetics, which could improve our understanding of the pathogenic mechanisms underlying neurodegeneration in HD.

Dr. Sybille Krauss
Guest Editor


Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. Papers will be published continuously (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

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  • neurodegeneration
  • Huntington's disease
  • polyglutamine proteins
  • CAG repeats
  • aggregation-prone proteins

Published Papers (3 papers)

by ,  and
Brain Sci. 2013, 3(4), 1588-1596; doi:10.3390/brainsci3041588
Received: 22 October 2013; in revised form: 1 November 2013 / Accepted: 12 November 2013 / Published: 20 November 2013
Show/Hide Abstract | PDF Full-text (806 KB) | HTML Full-text | XML Full-text

by  and
Brain Sci. 2014, 4(1), 91-122; doi:10.3390/brainsci4010091
Received: 10 January 2014; in revised form: 6 February 2014 / Accepted: 18 February 2014 / Published: 3 March 2014
Show/Hide Abstract | PDF Full-text (331 KB) | HTML Full-text | XML Full-text
abstract graphic

by ,  and
Brain Sci. 2014, 4(1), 202-219; doi:10.3390/brainsci4010202
Received: 2 October 2013; in revised form: 18 February 2014 / Accepted: 19 February 2014 / Published: 24 March 2014
Show/Hide Abstract | PDF Full-text (489 KB) | HTML Full-text | XML Full-text

Last update: 4 November 2013

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