Huntington's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Huntington's Disease, including details on genetics, causes, symptoms, treatment. | ||||||
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The processing of emotion in patients with Huntington's disease: variability and differential deficits in disgust.Hayes CJ, Stevenson RJ, Coltheart M Macquarie Centre for Cognitive Science, Sydney, New South Wales, Australia. BACKGROUND: Some studies of emotion recognition in Huntington's disease (HD) have not supported early reports of selective impairment in the recognition of facial expressions of disgust. This inconsistency could imply that loss of disgust is not a feature of all patients with this disease. OBJECTIVES: This study examined whether disproportionate impairment in the recognition of disgust was present in some HD patients and not in others. Second, we examined whether patients unable to recognize facial disgust had parallel impairments in other aspects of the emotion. METHOD: Fourteen HD patients and 14 age-matched healthy controls and education-matched healthy controls were first assessed on facial emotion recognition, with follow-up of individual-level analyses on patients D.W. and M.J. RESULTS: Although the group-level analyses revealed a broad profile of impaired recognition of negative emotions, individual-level analyses revealed a selective impairment of disgust in 47% of HD patients and of fear in 13%. Cross-modal impairments were only present for disgust, and then only in D.W. and M.J., who were unable to recognize disgust faces and had differential deficits on other emotion tasks: auditory recognition of vocal disgust expressions, matching the label "disgust" to a picture of a disgusting scene, and semantic knowledge of disgust elicitors. CONCLUSION: The findings support the view that impairment in the recognition of disgusted facial expressions may reflect processes involving the central aspects of disgust knowledge. Published 9 December 2009 in Cogn Behav Neurol, 22(4): 249-57. Articles on Huntington's Disease published 3 December 2009: Test-retest reliability of saccadic measures in subjects at risk for Huntington disease. Invest Ophthalmol Vis Sci, 50(12): 5707-11. PURPOSE: Abnormalities in saccades appear to be sensitive and specific biomarkers in the prediagnostic stages of Huntington disease (HD). The goal of this study was to evaluate test-retest reliability of saccadic measures in prediagnostic carriers of the HD gene expansion (PDHD) and normal controls (NC). METHODS: The study sample included 9 PDHD and 12 NC who completed two study visits within an approximate 1-month interval. At the first visit, all participants completed a uniform clinical ... [Abstract] [Full-text] Articles on Huntington's Disease published 30 November 2009: Overexpression of F(0)F(1)-ATP synthase alpha suppresses mutant huntingtin aggregation and toxicity in vitro. Biochem Biophys Res Commun, 390(4): 1294-8. Huntington's disease (HD) and other polyglutamine (polyQ) neurodegenerative diseases are characterized by neuronal accumulation of the disease protein, suggesting that the cellular ability to handle abnormal proteins is compromised. As a multi-subunit protein localized in the mitochondria of eukaryotic cells, the F(0)F(1)-ATP synthase alpha belongs to the family of stress proteins HSP60. Currently, mounting evidences indicate F(0)F(1)-ATP synthase alpha may play a role in neurodegenerative ... [Abstract] [Full-text] Articles on Huntington's Disease published 13 November 2009: Transcriptional dysregulation of coding and non-coding genes in cellular models of Huntington's disease. Biochem Soc Trans, 37: 1270-5. HD (Huntington's disease) is a late onset heritable neurodegenerative disorder that is characterized by neuronal dysfunction and death, particularly in the cerebral cortex and medium spiny neurons of the striatum. This is followed by progressive chorea, dementia and emotional dysfunction, eventually resulting in death. HD is caused by an expanded CAG repeat in the first exon of the HD gene that results in an abnormally elongated polyQ (polyglutamine) tract in its protein product, Htt ... [Abstract] [Full-text] Articles on Huntington's Disease published 28 October 2009: Age-related behavioural phenotype and cellular characterisation of mice with progressive ablation of D1 dopamine receptor-expressing cells. Behav Brain Res, 206(1): 78-87. In this study we characterize the behavioural and cellular phenotype of mutant (MUT) mice with progressive loss of D1 dopamine receptor (Drd1a)-expressing cells. Adult [14-19 weeks] MUT mice showed intact working memory in the spontaneous alternation test but evidenced anxiety-like behaviour in the elevated plus maze and the light-dark test. The ethogram of mature adult MUT [average age 22 weeks] was compared with that of young adult MUT mice [average age 12 weeks]. While MUT mice evidenced ... [Abstract] [Full-text] Protective effect of hesperidin and naringin against 3-nitropropionic acid induced Huntington's like symptoms in rats: possible role of nitric oxide. Behav Brain Res, 206(1): 38-46. 3-Nitropropionic acid (3-NP) is a well known experimental model to study Huntington's disease (HD) and associated neuropsychiatric problems. Present study has been designed to explore the protective effects of hesperidin, naringin, and their nitric oxide mechanism (if any) against 3-nitropropionic acid induced neurotoxicity in rats. Systemic 3-nitropropionic acid (10 mg/kg) treatment for 14 days in rats significantly induced HD like symptoms in rats as indicated by reduced locomotor activity, ... [Abstract] [Full-text] Articles on Huntington's Disease published 20 October 2009: Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease. Neurology, 73(16): 1280-5. OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could affect disease severity and progression. METHODS: Using linear regression and mixed-effects models, the influence of mutant and normal CAG repeat sizes interaction was assessed on 1) age at onset in 921 patients with HD, 2) clinical severity and ... [Abstract] [Full-text] BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution. J Neurochem, 111(3): 801-7. Bcl-2-associated athanogene-1 (BAG1) is a multifunctional protein delivering chaperone-recognized unfolded substrates to the proteasome for degradation. It has been shown to be essential for proper CNS development in vivo, playing a crucial role in neuronal survival and differentiation. With regard to Huntington's disease, a sequestration of BAG1 into inclusion bodies and a neuroprotective effect in double transgenic mice have been reported. Here, we show that BAG1 reduces aggregation and ... [Abstract] [Full-text] Articles on Huntington's Disease published 5 October 2009: Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease. Brain Res Bull, 80(4): 242-7. Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that is caused by a pathological expansion of CAG repeats within the gene encoding for a 350 kD protein called huntingtin. This polyglutamine expansion within huntingtin is the causative factor in the pathogenesis of HD, however the underlying mechanisms have not been fully elucidated. Nonetheless, it is becoming increasingly clear that alterations in mitochondrial function play key roles in the pathogenic processes ... [Abstract] [Full-text] © 2005-2010 Huntington's Disease Research Today. All Rights Reserved. |
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