https://scholars.lib.ntu.edu.tw/handle/123456789/556539
標題: | Deficiency of nuclear receptor interaction protein leads to cardiomyopathy by disrupting sarcomere structure and mitochondrial respiration | 作者: | KAI-CHIEN YANG Chuang K.-W. Yen W.-S. Lin S.-Y. Chen H.-H. Chang S.-W. Lin Y.-S. Wu W.-L. Tsao Y.-P. WEN-PIN CHEN SHOW-LI CHEN |
公開日期: | 2019 | 卷: | 137 | 起(迄)頁: | 9-24 | 來源出版物: | Journal of Molecular and Cellular Cardiology | 摘要: | Background: Cardiomyopathy is a common and lethal complication in patients with limb-girdle muscular dystrophy (LGMD), one of the most prevalent forms of muscular dystrophy. The pathogenesis underlying LGMD-related cardiomyopathy remains unclear. NRIP (gene name DCAF6), a Ca2+-dependent calmodulin binding protein, was reduced in dystrophic muscles from LGMD patients. Mice lacking NRIP exhibit a myopathic phenotype resembling that in LGMD patients, making NRIP deficiency a potential culprit leading to cardiomyopathy. This study aimed to determine if NRIP deficiency leads to cardiomyopathy and to explore the underlying molecular mechanisms. Methods and results: NRIP expression was reduced in both human and mouse failing hearts. Muscle-specific NRIP knockout (MCK-Cre |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/556539 | ISSN: | 222828 | DOI: | 10.1016/j.yjmcc.2019.09.009 | SDG/關鍵字: | adenosine triphosphate; alpha actinin 2; carrier proteins and binding proteins; F actin; nicotinamide adenine dinucleotide; nicotinic acid; nuclear receptor interaction protein; protein CapZ; reactive oxygen metabolite; reduced nicotinamide adenine dinucleotide; transcriptome; unclassified drug; actin; actinin; adenosine triphosphate; calcium; nicotinamide adenine dinucleotide; nicotinic acid; protein binding; reactive oxygen metabolite; receptor interacting protein 140; adult; animal cell; animal experiment; animal model; animal tissue; Article; cardiac muscle cell; cardiomyopathy; clinical article; confocal microscopy; controlled study; gene expression; heart muscle contractility; human; human tissue; immunoprecipitation; in vivo study; male; mitochondrial respiration; mouse; nonhuman; priority journal; protein deficiency; protein expression; protein protein interaction; sarcomere; transcriptomics; transmission electron microscopy; animal; biological model; cardiac muscle; cardiomyopathy; cell respiration; chemistry; down regulation; drug effect; genetics; heart contraction; heart failure; heart mitochondrion; homeostasis; metabolism; pathology; pathophysiology; phenotype; protein domain; sarcomere; ultrastructure; Actinin; Actins; Adenosine Triphosphate; Animals; Calcium; Cardiomyopathies; Cell Respiration; Down-Regulation; Heart Failure; Homeostasis; Humans; Male; Mice; Mitochondria, Heart; Models, Biological; Myocardial Contraction; Myocardium; Myocytes, Cardiac; NAD; Niacin; Nuclear Receptor Interacting Protein 1; Phenotype; Protein Binding; Protein Domains; Reactive Oxygen Species; Sarcomeres |
顯示於: | 藥理學科所 |
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