scholarly journals Myocardial morphology in hypertrophic cardiomyopathy: the current state of the problem

2019 ◽  
Vol 0 (3) ◽  
Author(s):  
A. A. Balabai
2021 ◽  
Vol 28 ◽  
Author(s):  
Andreas Angelopoulos ◽  
Evangelos Oikonomou ◽  
Georgia Vogiatzi ◽  
Alexios Antonopoulos ◽  
Sotirios Tsalamandris ◽  
...  

Background: Hypertrophic Cardiomyopathy (HCM) is the most common inherited Cardiomyopathy. The hallmark of HCM is myocardial fibrosis that contributes to heart failure, arrhythmias and sudden cardiac death. Objective: Currently there are no reliable serum biomarkers for detection of myocardial fibrosis, while cardiac magnetic resonance (CMR) is an imaging technique to detect myocardial fibrosis. MicroRNAs (miRNAs) have been increasingly suggested as biomarkers in cardiovascular diseases. However, in HCM there is as yet no identified and verified specific circulating miRNA signature. Methods: We conducted a review of literature to identify the studies that indicate the possible roles of miRNAs in HCM. Results: From studies in transgenic mice with HCM, miR-1, -133 may identify HCM in the early asymptomatic phase. Human miR-29a could be used as a circulating biomarker for detection of both myocardial hypertrophy and fibrosis in HCM, while it could also have a possible additional role in discrimination of hypertrophic obstructive cardiomyopathy from non-obstructive HCM. Additionally, miR-29a-3p is associated with diffuse myocardial fibrosis in HCM while miR-1-3p could discriminate end-stage HCM from dilated cardiomyopathy and left ventricle dilation. Another role of miRNAs could also be the contribution in differential diagnosis between HCM and phenocopies. Moreover, miRNA-targeted therapy (miR-133 mimics) is promising in inhibiting cardiac hypertrophy but this is still in the early stages. Conclusion: A more reliable and specific signature of miRNAs is expected with forthcoming studies in samples from HCM patients and correlation of miRNAs with CMR and serum markers of fibrosis may implicate novel diagnostic and therapeutic pathways.


2017 ◽  
Vol 93 (1) ◽  
pp. 3-14 ◽  
Author(s):  
M. Sabater-Molina ◽  
I. Pérez-Sánchez ◽  
J.P. Hernández del Rincón ◽  
J.R. Gimeno

Global Heart ◽  
2019 ◽  
Vol 14 (3) ◽  
pp. 317 ◽  
Author(s):  
Hussein H. Khachfe ◽  
Hamza A. Salhab ◽  
Mohamad Y. Fares ◽  
Hassan M. Khachfe

2021 ◽  
Vol 33 (3) ◽  
pp. 235-243
Author(s):  
Gökhan Yigit ◽  
Bernd Wollnik

Abstract Hypertrophic cardiomyopathy (HCM) is a clinically heterogeneous cardiac disease that is mainly characterized by left ventricular hypertrophy in the absence of any additional cardiac or systemic disease. HCM is genetically heterogeneous, inherited mainly in an autosomal dominant pattern, and so far pathogenic variants have been identified in more than 20 genes, mostly encoding proteins of the cardiac sarcomere. Based on its variable penetrance and expressivity, pathogenicity of newly identified variants often remains unsolved, underlining the importance of cellular and tissue-based models that help to uncover causative genetic alterations and, additionally, provide appropriate systems for the analysis of disease hallmarks as well as for the design and application of new therapeutic strategies like drug screenings and genome/base editing approaches. Here, we review the current state of cellular and tissue-engineered models and provide future perspectives for personalized therapeutic strategies of HCM.


Author(s):  
P. V. Lednev ◽  
A. V. Stonogin ◽  
A. V. Lysenko ◽  
G. I. Salagaev ◽  
Yu. V. Belov

Author(s):  
G.D. Danilatos

Over recent years a new type of electron microscope - the environmental scanning electron microscope (ESEM) - has been developed for the examination of specimen surfaces in the presence of gases. A detailed series of reports on the system has appeared elsewhere. A review summary of the current state and potential of the system is presented here.The gas composition, temperature and pressure can be varied in the specimen chamber of the ESEM. With air, the pressure can be up to one atmosphere (about 1000 mbar). Environments with fully saturated water vapor only at room temperature (20-30 mbar) can be easily maintained whilst liquid water or other solutions, together with uncoated specimens, can be imaged routinely during various applications.


Author(s):  
C. Barry Carter

This paper will review the current state of understanding of interface structure and highlight some of the future needs and problems which must be overcome. The study of this subject can be separated into three different topics: 1) the fundamental electron microscopy aspects, 2) material-specific features of the study and 3) the characteristics of the particular interfaces. The two topics which are relevant to most studies are the choice of imaging techniques and sample preparation. The techniques used to study interfaces in the TEM include high-resolution imaging, conventional diffraction-contrast imaging, and phase-contrast imaging (Fresnel fringe images, diffuse scattering). The material studied affects not only the characteristics of the interfaces (through changes in bonding, etc.) but also the method used for sample preparation which may in turn have a significant affect on the resulting image. Finally, the actual nature and geometry of the interface must be considered. For example, it has become increasingly clear that the plane of the interface is particularly important whenever at least one of the adjoining grains is crystalline.A particularly productive approach to the study of interfaces is to combine different imaging techniques as illustrated in the study of grain boundaries in alumina. In this case, the conventional imaging approach showed that most grain boundaries in ion-thinned samples are grooved at the grain boundary although the extent of this grooving clearly depends on the crystallography of the surface. The use of diffuse scattering (from amorphous regions) gives invaluable information here since it can be used to confirm directly that surface grooving does occur and that the grooves can fill with amorphous material during sample preparation (see Fig. 1). Extensive use of image simulation has shown that, although information concerning the interface can be obtained from Fresnel-fringe images, the introduction of artifacts through sample preparation cannot be lightly ignored. The Fresnel-fringe simulation has been carried out using a commercial multislice program (TEMPAS) which was intended for simulation of high-resolution images.


2005 ◽  
Vol 41 ◽  
pp. 205-218
Author(s):  
Constantine S. Mitsiades ◽  
Nicholas Mitsiades ◽  
Teru Hideshima ◽  
Paul G. Richardson ◽  
Kenneth C. Anderson

The ubiquitin–proteasome pathway is a principle intracellular mechanism for controlled protein degradation and has recently emerged as an attractive target for anticancer therapies, because of the pleiotropic cell-cycle regulators and modulators of apoptosis that are controlled by proteasome function. In this chapter, we review the current state of the field of proteasome inhibitors and their prototypic member, bortezomib, which was recently approved by the U.S. Food and Drug Administration for the treatment of advanced multiple myeloma. Particular emphasis is placed on the pre-clinical research data that became the basis for eventual clinical applications of proteasome inhibitors, an overview of the clinical development of this exciting drug class in multiple myeloma, and a appraisal of possible uses in other haematological malignancies, such non-Hodgkin's lymphomas.


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