نتایج جستجو برای: ژن myh7

تعداد نتایج: 16107  

Journal: :European Heart Journal 2022

Abstract Background Variants in MYH7 are responsible for disease 1–5% of patients with dilated cardiomyopathy (DCM); however, the clinical characteristics and natural history MYH7-related DCM poorly described. Objectives We sought to determine phenotype prognosis DCM. also evaluated influence variant location on phenotypic expression. Methods studied data from 147 individuals DCM-causing varian...

2007
Miriam Revera Lize Van Der Merwe Marshall Heradien Althea Goosen Paul A Brink Valerie A Corfield Johanna C Moolman-Smook

BACKGROUND The clinical profile and prognosis of patients with hypertrophic cardiomyopathy, a primary cardiac muscle disease caused mostly by mutations in sarcomeric protein-encoding genes, have been linked to particular disease-causing mutations in the past. However, such associations are often based on cross-sectional observations, as longitudinal studies of the progression of the disease in ...

2016
Shota Sasagawa Yuhei Nishimura Shiko Okabe Soichiro Murakami Yoshifumi Ashikawa Mizuki Yuge Koki Kawaguchi Reiko Kawase Ryuji Okamoto Masaaki Ito Toshio Tanaka

Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy and is associated with a number of potential outcomes, including impaired diastolic function, heart failure, and sudden cardiac death. Various etiologies have been described for HCM, including pressure overload and mutations in sarcomeric and non-sarcomeric genes. However, the molecular pathogenesis of HCM remain...

Journal: :Cardiogenetics 2022

Cardiomyopathies (CMPs) are a heterogeneous group of diseases, frequently genetic, affecting the heart muscle. The symptoms range from asymptomatic to dyspnea, arrhythmias, syncope, and sudden cardiac death. This study is focused on MYH7 (beta-myosin heavy chain), as this gene commonly mutated in cardiomyopathy patients. Due high combined prevalence variants severe health outcomes, it one most ...

Journal: :Cardiovascular research 2014
E Rosalie Witjas-Paalberends Ahmet Güçlü Tjeerd Germans Paul Knaapen Hendrik J Harms Alexa M C Vermeer Imke Christiaans Arthur A M Wilde Cris Dos Remedios Adriaan A Lammertsma Albert C van Rossum Ger J M Stienen Marjon van Slegtenhorst Arend F Schinkel Michelle Michels Carolyn Y Ho Corrado Poggesi Jolanda van der Velden

AIMS Disease mechanisms regarding hypertrophic cardiomyopathy (HCM) are largely unknown and disease onset varies. Sarcomere mutations might induce energy depletion for which until now there is no direct evidence at sarcomere level in human HCM. This study investigated if mutations in genes encoding myosin-binding protein C (MYBPC3) and myosin heavy chain (MYH7) underlie changes in the energetic...

2016
YUE ZHAO HONG CAO YINDI SONG YUE FENG XIAOXUE DING MINGJIE PANG YUNMEI ZHANG HONG ZHANG JIAHUAN DING XUESHAN XIA

Inherited cardiomyopathy is the major cause of sudden cardiac death (SCD) and heart failure (HF). The disease is associated with extensive genetic heterogeneity; pathogenic mutations in cardiac sarcomere protein genes, cytoskeletal protein genes and nuclear envelope protein genes have been linked to its etiology. Early diagnosis is conducive to clinical monitoring and allows for presymptomatic ...

Journal: :Cardiovascular research 2013
E Rosalie Witjas-Paalberends Nicoletta Piroddi Kelly Stam Sabine J van Dijk Vasco Sequeira Oliviera Claudia Ferrara Beatrice Scellini Mark Hazebroek Folkert J ten Cate Marjon van Slegtenhorst Cris dos Remedios Hans W M Niessen Chiara Tesi Ger J M Stienen Stephane Heymans Michelle Michels Corrado Poggesi Jolanda van der Velden

AIMS Familial hypertrophic cardiomyopathy (HCM), frequently caused by sarcomeric gene mutations, is characterized by cellular dysfunction and asymmetric left-ventricular (LV) hypertrophy. We studied whether cellular dysfunction is due to an intrinsic sarcomere defect or cardiomyocyte remodelling. METHODS AND RESULTS Cardiac samples from 43 sarcomere mutation-positive patients (HCMmut: mutatio...

Journal: :Circulation research 2002
Edward Blair Charles Redwood Marisa de Jesus Oliveira J C Moolman-Smook Paul Brink V A Corfield Ingegerd Ostman-Smith Hugh Watkins

Familial hypertrophic cardiomyopathy (HCM) is caused by mutations in 9 sarcomeric protein genes. The most commonly affected is beta-myosin heavy chain (MYH7), where missense mutations cluster in the head and neck regions and directly affect motor function. Comparable mutations have not been described in the light meromyosin (LMM) region of the myosin rod, nor would these be expected to directly...

Journal: :Clinical chemistry 2011
María Palacín Julián R Reguero María Martín Beatriz Díaz Molina César Morís Victoria Alvarez Eliecer Coto

MicroRNAs (miRNAs) regulate cardiac growth and conduction and play an important role in cardiac diseases (1 ). Several miRNAs are differentially produced in cardiac hypertrophic tissue, compared with normal tissue, and may contribute to the development of cardiomyocyte hypertrophy (2, 3 ). Hypertrophic cardiomyopathy (HCM) is frequently familial and caused by mutations in sarcomeric genes. To o...

Journal: :Circulation: Cardiovascular Genetics 2011

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