FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3105536308> ?p ?o ?g. }

• W3105536308 endingPage "273" @default.
• W3105536308 startingPage "237" @default.
• W3105536308 abstract "Abstract Sarcomeric cardiomyopathies are cardiovascular disorders that are caused by defects in genes encoding sarcomeric proteins. Depending on the location of the mutation within the gene, the disease phenotype manifests in different types of cardiac disorders, including hypertrophic and dilated cardiomyopathy, although mutations within the same gene can result in different phenotypes. Additionally, variability in penetrance and expressivity may result in a heterogeneous disease phenotype, not only across unrelated families but also within the same family. Hence, studies are needed to unravel both the initial and progressive changes initiated by sarcomere mutations, which will aid in better understanding how mutations in the same gene can lead to different phenotypes. As heart samples of the patients are poorly available, generation of cardiomyocytes from patient-specific induced pluripotent stem cells (iPSCs) provides a unique opportunity to investigate the pathophysiology of the mutation and to gain new insights into the underlying mechanisms. In this chapter, we review the emerging knowledge within the growing field of modeling sarcomeric cardiomyopathy with human iPSC-derived cardiomyocytes. These iPSC-based studies employed diverse systems ranging from single cell techniques to 3D engineered heart tissue. Where possible, we also compare the results of different studies with patient phenotypes. Furthermore, we discuss the prospects and limitations of the current state-of-the-art iPSC technology. We conclude that application of patient- and disease-specific iPSCs and differentiation into cardiomyocytes certainly offers the advantage to recapitulate the disease phenotype and will continue to provide critical knowledge about the underlying mechanisms of sarcomeric cardiomyopathies. This may allow development of disease- and patient-specific drug therapies for these devastating diseases to improve cardiac function and to prevent progressive cardiac remodeling." @default.
• W3105536308 created "2020-11-23" @default.
• W3105536308 creator A5014677921 @default.
• W3105536308 creator A5076376167 @default.
• W3105536308 date "2020-01-01" @default.
• W3105536308 modified "2023-10-14" @default.
• W3105536308 title "iPSCs for modeling of sarcomeric cardiomyopathies" @default.
• W3105536308 cites W145908001 @default.
• W3105536308 cites W1898753515 @default.
• W3105536308 cites W1899708338 @default.
• W3105536308 cites W1963682290 @default.
• W3105536308 cites W1969832018 @default.
• W3105536308 cites W1970944947 @default.
• W3105536308 cites W1972328168 @default.
• W3105536308 cites W1972667905 @default.
• W3105536308 cites W1977025496 @default.
• W3105536308 cites W1977354800 @default.
• W3105536308 cites W1989044657 @default.
• W3105536308 cites W1991507611 @default.
• W3105536308 cites W1994619997 @default.
• W3105536308 cites W1999251970 @default.
• W3105536308 cites W2001045428 @default.
• W3105536308 cites W2003311702 @default.
• W3105536308 cites W2007185913 @default.
• W3105536308 cites W2012646046 @default.
• W3105536308 cites W2026182775 @default.
• W3105536308 cites W2029062125 @default.
• W3105536308 cites W2030312576 @default.
• W3105536308 cites W2030462532 @default.
• W3105536308 cites W2035312069 @default.
• W3105536308 cites W2046110995 @default.
• W3105536308 cites W2057982492 @default.
• W3105536308 cites W2059060273 @default.
• W3105536308 cites W2061892272 @default.
• W3105536308 cites W2065572778 @default.
• W3105536308 cites W2066390373 @default.
• W3105536308 cites W2068698281 @default.
• W3105536308 cites W2075469935 @default.
• W3105536308 cites W2076034570 @default.
• W3105536308 cites W2079592630 @default.
• W3105536308 cites W2082231490 @default.
• W3105536308 cites W2082936028 @default.
• W3105536308 cites W2083599207 @default.
• W3105536308 cites W2088152986 @default.
• W3105536308 cites W2089085237 @default.
• W3105536308 cites W2091736982 @default.
• W3105536308 cites W2091831926 @default.
• W3105536308 cites W2095487467 @default.
• W3105536308 cites W2096299114 @default.
• W3105536308 cites W2099302775 @default.
• W3105536308 cites W2104790299 @default.
• W3105536308 cites W2105276369 @default.
• W3105536308 cites W2105955220 @default.
• W3105536308 cites W2107752854 @default.
• W3105536308 cites W2108710722 @default.
• W3105536308 cites W2108892010 @default.
• W3105536308 cites W2108903695 @default.
• W3105536308 cites W2109136976 @default.
• W3105536308 cites W2112484130 @default.
• W3105536308 cites W2115177875 @default.
• W3105536308 cites W2117224856 @default.
• W3105536308 cites W2121245479 @default.
• W3105536308 cites W2121902325 @default.
• W3105536308 cites W2125987139 @default.
• W3105536308 cites W2126611898 @default.
• W3105536308 cites W2127423627 @default.
• W3105536308 cites W2129677512 @default.
• W3105536308 cites W2129794513 @default.
• W3105536308 cites W2129992226 @default.
• W3105536308 cites W2131645973 @default.
• W3105536308 cites W2133193948 @default.
• W3105536308 cites W2134406150 @default.
• W3105536308 cites W2135921719 @default.
• W3105536308 cites W2137542902 @default.
• W3105536308 cites W2137802443 @default.
• W3105536308 cites W2138581343 @default.
• W3105536308 cites W2138977668 @default.
• W3105536308 cites W2139006361 @default.
• W3105536308 cites W2142046859 @default.
• W3105536308 cites W2142388130 @default.
• W3105536308 cites W2142533013 @default.
• W3105536308 cites W2145372543 @default.
• W3105536308 cites W2148220839 @default.
• W3105536308 cites W2149847832 @default.
• W3105536308 cites W2151433089 @default.
• W3105536308 cites W2152224560 @default.
• W3105536308 cites W2152272372 @default.
• W3105536308 cites W2153354609 @default.
• W3105536308 cites W2156433522 @default.
• W3105536308 cites W2157085976 @default.
• W3105536308 cites W2157842394 @default.
• W3105536308 cites W2158081637 @default.
• W3105536308 cites W2159453909 @default.
• W3105536308 cites W2160235532 @default.
• W3105536308 cites W2163068618 @default.
• W3105536308 cites W2167730267 @default.
• W3105536308 cites W2167989424 @default.
• W3105536308 cites W2174048979 @default.

• next