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T1  - Modeling of LMNA-Related Dilated Cardiomyopathy Using Human Induced Pluripotent Stem Cells 
SN  -  /  
UR  - http://hdl.handle.net/10138/305157 
T3  -  
A1  - Shah, Disheet; Virtanen, Laura; Prajapati, Chandra; Kiamehr, Mostafa; Gullmets, Josef; West, Gun; Kreutzer, Joose; Pekkanen-Mattila, Mari; Heliö, Tiina; Kallio, Pasi; Taimen, Pekka; Aalto-Setälä, Katriina 
A2  -  
PB  -  
Y1  - 2019 
LA  - eng 
AB  - Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure and heart transplantation. A portion of familial DCM is due to mutations in the LMNA gene encoding the nuclear lamina proteins lamin A and C and without adequate treatment these patients have a poor prognosis. To get better insights into pathobiology behind this disease, we focused on modeling LMNA-related DCM using human induced pluripotent stem cell derived cardiomyocytes (hiPSC-CM). Primary skin fibroblasts from DCM pa... 
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KW  - dilated cardiomyopathy; LMNA; Lamin A; C; induced pluripotent stem cell; hypoxia; microelectrode array and calcium imaging; TO-BEAT VARIABILITY; SARCOPLASMIC-RETICULUM; VENTRICULAR MYOCYTES; HEART-FAILURE; MUTATION; GENE; REPOLARIZATION; STIMULATION; EXPRESSION; HYPOXIA; 1182 Biochemistry, cell and molecular biology; 3111 Biomedicine; 3121 General medicine, internal medicine and other clinical medicine 
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