نتایج جستجو برای: ژن myh7

تعداد نتایج: 16107  

Journal: :Journal of medical genetics 2000
J F Forissier P Richard S Briault C Ledeuil O Dubourg B Charbonnier L Carrier C Moraine G Bonne M Komajda K Schwartz B Hainque

Familial hypertrophic cardiomyopathy is a genetically and phenotypically heterogeneous disease caused by mutations in seven sarcomeric protein genes. It is known to be transmitted as an autosomal dominant trait with rare de novo mutations.A French family in which two members are affected by hypertrophic cardiomyopathy was clinically screened with electrocardiography and echocardiography. Geneti...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2009
Thomas Z Armel Leslie A Leinwand

Myosin storage myopathy (MSM) is a congenital myopathy characterized by the presence of subsarcolemmal inclusions of myosin in the majority of type I muscle fibers, and has been linked to 4 mutations in the slow/cardiac muscle myosin, beta-MyHC (MYH7). Although the majority of the >230 disease causing mutations in MYH7 are located in the globular head region of the molecule, those responsible f...

Journal: :Journal of medical genetics 1999
P Richard R Isnard L Carrier O Dubourg Y Donatien B Mathieu G Bonne F Gary P Charron M Hagege M Komajda K Schwartz B Hainque

Familial hypertrophic cardiomyopathy is a genetically heterogeneous autosomal dominant disease, caused by mutations in several sarcomeric protein genes. So far, seven genes have been shown to be associated with the disease with the beta-myosin heavy chain (MYH7) and the cardiac myosin binding protein C (MYBPC3) genes being the most frequently involved. We performed electrocardiography (ECG) and...

Journal: :Circulation. Cardiovascular imaging 2017
Adaya Weissler-Snir Waseem Hindieh Christiane Gruner Dana Fourey Evan Appelbaum Ethan Rowin Melanie Care John R Lesser Tammy S Haas James E Udelson Warren J Manning Iacopo Olivotto Benedetta Tomberli Barry J Maron Martin S Maron Andrew M Crean Harry Rakowski Raymond H Chan

BACKGROUND The 2 most commonly affected genes in hypertrophic cardiomyopathy (HCM) are MYH7 (β-myosin heavy chain) and MYBPC3 (β-myosin-binding protein C). Phenotypic differences between patients with mutations in these 2 genes have been inconsistent. Scarce data exist on the genotype-phenotype association as assessed by tomographic imaging using cardiac magnetic resonance imaging. METHODS AN...

2014
Marijke Wasielewski Karin Y van Spaendonck-Zwarts Nico-Derk L Westerink Jan D H Jongbloed Aleida Postma Jourik A Gietema J Peter van Tintelen Maarten P van den Berg

OBJECTIVE Anthracyclines are successfully used in cancer treatment, but their use is limited by their cardiotoxic side effects. Several risk factors for anthracycline-associated cardiomyopathy (AACM) are known, yet the occurrence of AACM in the absence of these known risk factors suggests that other factors must play a role. The purpose of this study was to evaluate whether a genetic predisposi...

2017
Lucia Binó Jiřina Procházková Katarzyna Anna Radaszkiewicz Jan Kučera Jana Kudová Jiří Pacherník Lukáš Kubala

The potentiation of the naturally limited regenerative capacity of the heart is dependent on an understanding of the mechanisms that are activated in response to pathological conditions such as hypoxia. Under these conditions, the expression of genes suggested to support cardiomyocyte survival and heart adaptation is triggered. Particularly important are changes in the expression of myosin heav...

Journal: :Journal of clinical pathology 2005
B Yu N A Sawyer M Caramins Z G Yuan R B Saunderson R Pamphlett D R Richmond R W Jeremy R J Trent

AIMS To evaluate the usefulness of denaturing high performance liquid chromatography (DHPLC) as a high throughput tool in: (1) DNA mutation detection in familial hypertrophic cardiomyopathy (FHC), and (2) single nucleotide polymorphism (SNP) discovery and validation in sporadic motor neurone disease (MND). METHODS The coding sequence and intron-exon boundaries of the cardiac beta myosin heavy...

1999
Pascale Richard Richard Isnard Lucie Carrier Olivier Dubourg Yves Donatien Bénédicte Mathieu Gisèle Bonne Françoise Gary Philippe Charron Albert Hagege Michel Komajda Ketty Schwartz Bernard Hainque

Familial hypertrophic cardiomyopathy is a genetically heterogeneous autosomal dominant disease, caused by mutations in several sarcomeric protein genes. So far, seven genes have been shown to be associated with the disease with the â-myosin heavy chain (MYH7) and the cardiac myosin binding protein C (MYBPC3) genes being the most frequently involved. We performed electrocardiography (ECG) and ec...

Journal: :Developmental cell 2009
Eva van Rooij Daniel Quiat Brett A Johnson Lillian B Sutherland Xiaoxia Qi James A Richardson Robert J Kelm Eric N Olson

Myosin is the primary regulator of muscle strength and contractility. Here we show that three myosin genes, Myh6, Myh7, and Myh7b, encode related intronic microRNAs (miRNAs), which, in turn, control muscle myosin content, myofiber identity, and muscle performance. Within the adult heart, the Myh6 gene, encoding a fast myosin, coexpresses miR-208a, which regulates the expression of two slow myos...

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