A
montage of three images of single striatal neurons transfected with a
disease-associated version of huntingtin, the protein that causes Huntington's
disease. Nuclei of untransfected neurons are seen in the background (blue). The
neuron in the center (yellow) contains an abnormal intracellular accumulation
of huntingtin called an inclusion body (orange). Credit: Wikipedia/ Creative
Commons Attribution 3.0 Unported license
Huntington's
disease is an hereditary disorder of the nervous system caused by a faulty gene
on chromosome four. The faulty gene leads to cell death in neurons in the brain
resulting in gradual physical, mental and emotional changes, and ultimately
death. Those born to a parent with Huntington's disease have a 50:50 chance of
developing it, and there is currently no cure.
Scientists
around the world are working to find effective
therapies for Huntington's disease. While the disease is caused by a
single gene mutation, the manifestation of the disease at a molecular level is
nevertheless highly complex and involves multiple cellular
processes. In research, this complexity has resulted in huge and
diverse sets of data which in turn pose considerable challenges to researchers
in collating, analysing and cross-referencing varied data sources.
An
international team of researchers, bioinformaticians and biostatisticians has
developed the first freely available data network for scientists researching
this debilitating and fatal condition, called HDNetDB. In a paper published in Scientific
Reports, the HDNetDB team outlines the challenges facing those
investigating Huntington's disease, explains how HDNetDB works and illustrates
its effectiveness by discovering potential drug targets, which might help to
prevent the neuronal cell death
observed in Huntington's disease. A unique feature of this new resource is that
it links various cellular processes in a molecular network and provides a more
holistic view of the disease.
The
team also states that HDNetDB could form the blueprint for similar data
networks relating to other specific conditions.
HDNetDB
allows users to obtain, visualise and prioritise molecular interaction networks
using Huntington's disease-related gene expression and other types of data
obtained from human samples and other sources.
Leading
the project from the University of Plymouth is Matthias Futschik, Professor in
Bioinformatics at the University's School of Biomedical and Healthcare
Sciences.
He
commented: "Huntington's disease is especially devastating for affected
families, since we can nowadays exactly predict who will be affected later in
life, but we cannot yet provide any cure. So, it is important that those
scientists seeking effective therapies are not hampered by poor access to the vast
array of data related to the disease and can connect the different pathological
mechanisms. In HDNetDB we have developed a computational tool which allows
those scientists free and open access, helping them to identify new molecular
targets for the development of new drug strategies for the disease.
We
believe that our approach can be applied to other neurodegenerative disorders
such as Alzheimer's and Parkinson's
disease and could facilitate the development of new therapies for these
diseases too."
More
information: Ravi Kiran Reddy Kalathur et al, HDNetDB: A
Molecular Interaction Database for Network-Oriented Investigations into
Huntington's Disease, Scientific Reports (2017). DOI: 10.1038/s41598-017-05224-0
Journal reference: Scientific
Reports
Provided by: University of
Plymouth
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