The paper reviewed here is ‘Beyond the Brain: widespread pathology in Huntington’s Disease’ by van der Burg and colleagues.The article is written by a group from the Swedish Lund Institute Neuronal survival unit and is classed as a ‘personal view’. However there is a small green box towards the end of the article which contains details of the search strategy and selection criteria which thus allows the reader to gain a better understanding of how the article was orginally constructed as well as providing a useful starting point for someone interested in updating the article. The article focuses on non-neuronal aspects of Huntington’s Disease (HD) in keeping with the expression of Huntingtin in such tissues. The article begins with a look at some of the posited functions of the Huntingtin protein which sets the scene for an exploration of non-neuronal manifestations of HD. There is a look at some of the processes that may contribute to weight loss including possible alterations to Insulin processing and adipocytes although much of the work here is in murine models. The potential impact of mutated Huntingtin on gene expression in myocytes, the expression of Huntingtin in cardiac myocytes, possible actions on osteoblasts or osteoclasts and altered immune response are all considered. The authors then return to the question of the cellular basis of these observations before looking at how such a discussion may inform the search for biomarkers and novel therapeutic paradigms. This is a concise review which references 110 papers and thus provides the reader interested in this area with a useful starting point for their investigations.
Van der Burg J M M, Bjorkqvist M and Brundin P. Beyond the brain: widespread pathology in Huntington’s Disease. Lancet Neurology. 2009. 8. 765-74.
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