RESEARCH ARTICLE

Making (anti-) sense out o lek Huntington disease (HD) is an autosomal dominant neu- will develop HD, whereas people with 36 to 39 repeats Evers et al. Molecular Neurodegeneration (2015) 10:21 DOI 10.1186/s13024-015-0018-7plasm of cells are a hallmark of the disease [5].Albinusdreef 2, Leiden 2333ZA, the Netherlands Full list of author information is available at the end of the articlerodegenerative disorder, characterized by motor, psychi-atric and cognitive symptoms [1]. HD is caused by a CAG repeat expansion in the first exon of the HTT gene on chromosome 4p16, resulting in an expanded polyglu-tamine (polyQ) tract at the N-terminus of the huntingtin show reduced penetrance [2,3]. The mean disease onset lies between 30 and 50 years of age (adult-onset HD). HD patients carrying more than 50 CAGs will have a disease onset typically below 21 years of age (juvenile HD) [1]. The major neuropathology in HD occurs in the striatum and cerebral cortex but degeneration is seen throughout the brain as the disease progresses [4] and insoluble protein aggregates in the nucleus and cyto