[Huntington's disease].

Lijec Vjesn

Hrvatski institut za istrazivanje mozga, Medicinski fakultet Sveucilista u Zagrebu.

Published: April 2013

Huntington's disease and/or chorea (HD) is autosomal dominant neurodegenerative disease that never skips generations. The first description was provided by George Huntington in the year 1872. Its prevalence in the world is 8-10 per 100000 inhabitants and in Croatia 4.46 per 100 000 inhabitants. It starts between 30 and 50 years of age and ends after 15-20 years with death. It is a disease of CAG triplet repeats and is characterized by poliglutamine repeats. The number of CAG trinucleotid repeats correlates with the age of the onset of the first symptoms, as well as with the clinical picture. The selective therapy for Huntington's chorea still does not exists and for symptomatic treatment the blockers of dopamine have turned out to be the most useful.

Download full-text PDF

Source

Publication Analysis

Top Keywords

[huntington's disease]
4
disease] huntington's
4
huntington's disease
4
disease and/or
4
and/or chorea
4
chorea autosomal
4
autosomal dominant
4
dominant neurodegenerative
4
neurodegenerative disease
4
disease skips
4

Similar Publications

Drug Development.

Alzheimers Dement

December 2024

iCBR - Coimbra Institute for Clinical and Biomedical Research, Faculty of Medicine, University of Coimbra, Coimbra, Coimbra, Portugal; Institute of Pharmacology and Experimental Therapeutics, Faculty of Medicine, University of Coimbra, Coimbra, Coimbra, Portugal; CIBB - Center for Innovative Biomedicine and Biotechnology, University of Coimbra, Coimbra, Coimbra, Portugal; Institute of Interdisciplinary Research (IIIUC), University of Coimbra, Coimbra, Coimbra, Portugal; CNC-UC - Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Coimbra, Portugal.

Background: Cardiometabolic diseases, such as type 2 diabetes, hypertension, dyslipidemia or obesity, constitute major causes of mortality and morbidity worldwide, especially among middle-aged individuals. The increasing incidence and association with aging and lifestyle, render the cardiometabolic diseases a societal concern. This is further reinforced by their association with an increased risk of cognitive impairment and neurodegenerative diseases (namely dementia and Alzheimer's disease (AD)).

View Article and Find Full Text PDF

Effects and mechanisms of computerized cognitive training in Huntington's disease: protocol for a pilot study.

Neurodegener Dis Manag

January 2025

Turner Institute for Brain & Mental Health, School of Psychological Sciences, Faculty of Medicine, Nursing & Health Sciences, 18 Innovation Walk, Monash University, Clayton VIC 3800, Australia.

Huntington's disease (HD) causes progressive cognitive decline, with no available treatments. Computerized cognitive training (CCT) has shown efficacy in other populations, but its effects in HD are largely unknown. This pilot study will explore the effects and neural mechanisms of CCT in HD.

View Article and Find Full Text PDF

Intermittent Fasting Enhances Motor Coordination Through Myelin Preservation in Aged Mice.

Aging Cell

January 2025

Department of Neurology, Songjiang Research Institute, Shanghai Key Laboratory of Emotions and Affective Disorders, Songjiang Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Integrating dietary interventions have been extensively studied for their health benefits, such as Alzheimer's disease, Huntington's disease, and aging. However, it is necessary to fully understand the mechanisms of long-term effects and practical applications of these dietary interventions for health. A 10-week intermittent fasting (IMF) regimen was implemented on the aging animals in the current study.

View Article and Find Full Text PDF

Background: Mitochondrial dysfunction and neuronal damage are major sign of cytopathology in Huntington's disease (HD), a neurodegenerative disease. Ubiquitin specific peptidase 11 (USP11) is a deubiquitinating enzyme involved in various physiological processes through regulating protein degradation. However, its specific role in HD is unclear.

View Article and Find Full Text PDF

Huntington's disease (HD), a neurodegenerative disease, affects approximately 30,000 people in the United States, with 200,000 more at risk. Mitochondrial dysfunction caused by mutant huntingtin (mHTT) drives early HD pathophysiology. mHTT binds the translocase of mitochondrial inner membrane (TIM23) complex, inhibiting mitochondrial protein import and altering the mitochondrial proteome.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!