Cardiac Dysfunction in the BACHD Mouse Model of Huntington’s Disease

• DOI: 10.1371/journal.pone.0147269
• Publication Date: 2016-01-25T19:12:25Z
• Authors: Analyne M. Schroeder; Huei Bin Wang; Saemi Park; Maria C. Jordan; Fuying Gao; Giovanni Coppola; Michael C. Fishbein; Kenneth P. Roos; Cristina A. Ghiani; Christopher S. Co...
• Keywords: Huntington's Disease; Aging; General Science & Technology; Science; Heart Ventricles; Left; Apoptosis; Cardiomegaly; Mice, Transgenic; Nerve Tissue Proteins; Neurodegenerative; Inbred C57BL; Cardiovascular; Real-Time Polymerase Chain Reaction; Transgenic; Mice; 03 medical and health sciences; Rare Diseases; Ventricular Dysfunction; Genetics; 2.1 Biological and endogenous factors; Animals; Aetiology; Ultrasonography; Huntingtin Protein; 0303 health sciences; Animal; Gene Expression Profiling; Q; Neurosciences; R; Isoproterenol; Adrenergic beta-Agonists; Fibrosis; Myocardial Contraction; Brain Disorders; 3. Good health; Mice, Inbred C57BL; Disease Models, Animal; Heart Disease; Huntington Disease; Tissue Array Analysis; Disease Models; Medicine; Trinucleotide Repeat Expansion; Multiplex Polymerase Chain Reaction; Research Article

While Huntington's disease (HD) is classified as a neurological disorder, HD patients exhibit high incidence of cardiovascular events leading to heart failure and death. In this study, we sought better understand the phenotype using BACHD mouse model. The age-related decline in function was assessed by echocardiograms, electrocardiograms, histological microarray analysis. We found that structural functional differences between WT hearts start at 3 months age continue throughout life. aged mice develop cardiac fibrosis ultimately apoptosis. exhibited adaptive physiological changes chronic isoproterenol treatment; however, medication exacerbated fibrotic lesions heart. Gene expression analysis indicated strong tilt toward apoptosis young mutant well genes involved cellular metabolism proliferation. With age, number with altered increased large occurring disease, metabolism, transport clusters. model exhibits early progress may provide an explanation for higher risk HD.