...Jessica Marotta; Carla Piano; Valerio Brunetti; Danilo Genovese; Anna Rita Bentivoglio; Paolo Calabresi; Pietro Cortelli; Giacomo Della Marca Introduction: Autonomic dysfunction has been reported as one of nonmotor manifestations of both presymptomatic and manifest Huntington’s disease (HD...

...Beatrice Heim; Marina Peball; Carsten Saft; Sarah M. von Hein; Johanna M. Piater; Philipp Ellmerer; Klaus Seppi; Atbin Djamshidian-Tehrani Objective: We aimed to investigate costly punishment in patients with Huntington’s disease (HD). Background: HD is an autosomal dominant neurodegenerative...

...Beatrice Heim; Dora Valent; Federico Carbone; Sabine Spielberger; Florian Krismer; Atbin Djamshidian-Tehrani; Klaus Seppi Objective: The aim of this pilot study was to investigate change of olfactory functions in Huntington’s disease (HD). Background: HD is a neurodegenerative disease characterized...

...Petra Smatlikova; Georgina Askeland; Michaela Vaskovicova; Jiri Klima; Jan Motlik; Lars Eide; Zdenka Ellederová Background: Huntington’s disease (HD) is a devastating neurodegenerative disorder caused by CAG triplet expansions in the huntingtin gene. Oxidative stress is linked to HD pathology...

...Ana Rita Saldanha Ramos; Carolina Garrett Huntington's disease (HD) is an incurable, neurodegenerative disease, which manifests via a triad of progressive symptoms: motor impairment, psychiatric disorders, and cognitive decline. Conventionally, the HD diagnosis is based on the presence...

... Spacilova; Ryan Collins; Ian Blumenthal; Michael Talkowski; James F. Gusella; David S. Howland; Marian DiFiglia; Jan Motlik Background: Huntington's disease is induced by CAG expansion in a single gene coding the huntingtin protein. The mutated huntingtin (mtHtt) primarily causes degeneration of neurons...

...Erik van Duijn; Nanda Reedeker; Erik J. Giltay; Daniëlle Eindhoven; Raymund A.C. Roos; Rose C. van der Mast Background: Irritability, depression and apathy are frequently reported neuropsychiatric symptoms of Huntington's disease (HD). Objective: This study investigated the course of irritability...

...Imis Dogan; Simon B. Eickhoff; Jörg B. Schulz; N. Jon Shah; Angela R. Laird; Peter T. Fox; Kathrin Reetz Background: The neuropathological hallmark of Huntington's disease (HD) is progressive striatal loss starting several years prior to clinical onset. In the past decade, whole-brain magnetic...

... carriers of the Huntington's disease gene mutation (preHD). So far, structural changes have been found mainly in preHD approaching the estimated motor onset of the disease (i.e. less than 15 years from onset), whereas structural findings in preHD far from the estimated motor onset have been inconclusive...

...Rajat Sandhir; Aditi Sood; Arpit Mehrotra; Sukhdev S. Kamboj Mitochondrial dysfunction is a major event involved in the pathogenesis of Huntington’s disease (HD). The present study evaluates the role of N -acetyl- L -cysteine (NAC) in preventing mitochondrial dysfunctions in a 3-nitropropionic acid...

...Laszlo Bodai; Judit Pallos; Leslie Michels Thompson; J. Lawrence Marsh Huntingtin peptides with elongated polyglutamine domains, the root causes of Huntington’s disease, hinder histone acetylation, which leads to transcriptional dysregulation. However, the range of acetyltransferases interacting...

...Åsa Petersén; Sofia Hult; Deniz Kirik Huntington’s disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene. Although it is characterized by progressive motor impairments, cognitive changes and psychiatric disturbances are major components of the disease...

... mice Gene therapy Huntington’s disease Alzheimer’s disease Parkinson’s disease Diseases Therapeutic Strategies Neurodegenerative Dis 2005;2:220 231 DOI: 10.1159/000089629 Promises and Challenges in Developing RNAi as a Research Tool and Therapy for Neurodegenerative Diseases Xu Gang Xia Hongxia...