Special Issue "Molecular Mechanisms Underlying Huntington's Disease"
A special issue of Brain Sciences (ISSN 2076-3425).
Deadline for manuscript submissions: 31 March 2014
Dr. Sybille Krauss
German Center for Neurodegenerative Diseases (DZNE), Biomedical Center (BMZ1) - Building 344, Sigmund-Freud-Str. 25, 53127 Bonn, Germany
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
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.
Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are refereed through a peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Brain Sciences is an international peer-reviewed Open Access quarterly journal published by MDPI.
Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 500 CHF (Swiss Francs). English correction and/or formatting fees of 250 CHF (Swiss Francs) will be charged in certain cases for those articles accepted for publication that require extensive additional formatting and/or English corrections.
- Huntington's disease
- polyglutamine proteins
- CAG repeats
- aggregation-prone proteins
Article: Altered Neuronal Dynamics in the Striatum on the Behavior of Huntingtin Interacting Protein 14 (HIP14) Knockout Mice
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| Download PDF Full-text (806 KB) | View HTML Full-text | Download XML Full-text
Review: Monomeric, Oligomeric and Polymeric Proteins in Huntington Disease and Other Diseases of Polyglutamine Expansion
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| Download PDF Full-text (331 KB) | Download XML Full-text
Last update: 4 November 2013