ArticleJournal of Huntington's disease2012
Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease.
Article in Journal of Huntington's disease, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 37 citations in OpenAlex.
- Systemic Symptoms in Huntington's Disease: A Comprehensive Review.Movement disorders clinical practice · 2024Pooled it
- Sex-dependent mild cardiac dysfunction and neurometabolic abnormalities in a model of Huntington's disease.Disease models & mechanisms · 2026Article
- Concomitant pathologies and their impact on Huntington's disease. A brief review of current evidence.Journal of neural transmission (Vienna, Austria : 1996) · 2025Review
- Review
- Brain-Periphery Interactions in Huntington's Disease: Mediators and Lifestyle Interventions.International journal of molecular sciences · 2024Review
- Article
- A New Perspective on Huntington's Disease: How a Neurological Disorder Influences the Peripheral Tissues.International journal of molecular sciences · 2022Review
- Huntington's disease phenotypes are improved via mTORC1 modulation by small molecule therapy.PloS one · 2022Article
- Purine Nucleotides Metabolism and Signaling in Huntington's Disease: Search for a Target for Novel Therapies.International journal of molecular sciences · 2021Review
- Targeted Genetic Reduction of Mutant Huntingtin Lessens Cardiac Pathology in the BACHD Mouse Model of Huntington's Disease.Frontiers in cardiovascular medicine · 2021Article
- Progressive cardiac arrhythmias and ECG abnormalities in the Huntington's disease BACHD mouse model.Human molecular genetics · 2020Article
- Altered cerebrovascular response to acute exercise in patients with Huntington's disease.Brain communications · 2020Article
- Circadian dysfunction in the Q175 model of Huntington's disease: Network analysis.Journal of neuroscience research · 2019Article
- Do Disruptions in the Circadian Timing System Contribute to Autonomic Dysfunction in Huntington's Disease?The Yale journal of biology and medicine · 2019Review
- Cardiac mTORC1 Dysregulation Impacts Stress Adaptation and Survival in Huntington's Disease.Cell reports · 2018Article
- Neuro-Cardio Mechanisms in Huntington's Disease and Other Neurodegenerative Disorders.Frontiers in physiology · 2018Review
- Neurocardiovascular deficits in the Q175 mouse model of Huntington's disease.Physiological reports · 2017Article
- Metabolic profiling of presymptomatic Huntington's disease sheep reveals novel biomarkers.Scientific reports · 2017Article
- Motor phenotype is not associated with vascular dysfunction in symptomatic Huntington's disease transgenic R6/2 (160 CAG) mice.Scientific reports · 2017Article
- Huntington's Disease-Induced Cardiac Disorders Affect Multiple Cellular Pathways.Reactive oxygen species (Apex, N.C.) · 2016Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 2 countries.
Funding
Abstract
HD is a progressive genetic neurological disorder, characterized by motor as well as cognitive impairments. The gene carrying the mutation causing Huntington's disease (HD) is not brain specific, and there is increasing evidence for peripheral, as well as brain pathology in this disorder. Here, we used in vivo and ex vivo techniques to assess the cardiac function of mice transgenic for the HD mutation. Using magnetic resonance imaging (MRI) of the beating heart, we show that abnormalities previously reported in end-stage mice are present by mid-stages of the disease. We also found abnormalities that have not been hitherto reported, including changes in cardiac efficiency and a mechanical distortion of the beating heart. Using the Langendorff preparation, we show reduced coronary blood flow, impaired myocardial contractility and reduced left ventricular developed pressure in HD mouse hearts. Together, our findings suggest that there is significant pathology of the HD mouse heart, even by mid stages of disease. Previous clinical research has demonstrated that the risk of cognitive symptoms increases markedly in patients with heart failure. R6/2 mice show significant progressive cognitive abnormalities, so we hypothesize that cardiac pathology in the R6/2 mouse may contribute, not only to their progressive decline and death, but also to their cognitive dysfunction. We suggest that closer attention should be paid to cardiovascular symptoms in HD patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.