conklin lab

Coriell Cat. # GM25305

These cells were derived from a skin biopsy from an Asian female donor with a known A422T point mutation (HERG 1264 G>A) associated with Long QT Syndrome, Type 2.  An EKG reveals QT prolongation in iPSC-cardiomyocytes derived from this line.  The original patient fibroblasts were reprogrammed using retroviral methods with factors: KLF4, MYC (c-MYC), POU5F1 (OCT4), and SOX2. The iPSC line also has a normal karyotype (46 XX). Immunostaining and RT-PCR was carried out to confirm reprogramming to the pluripotent state. Its pluripotency and differentiation potential was characterized using teratoma formation, embryoid formation, and cardiomyocyte differentiation.

Publications:

Calcium transients closely reflect prolonged action potentials in iPSC models of inherited cardiac arrhythmia.  C. Ian Spencer, Shiro Baba, Kenta Nakamura, Ethan A. Hua, Marie A.F. Sears, Chi-cheng Fu, Jianhua Zhang, Sadguna Balijepalli, Kiichiro Tomoda, Yohei Hayashi, Paweena Lizarraga, Julianne Wojciak, Melvin M. Scheinman, Katriina Aalto-Setälä, Jonathan C. Makielski, Craig T. January, Kevin E. Healy, Timothy J. Kamp, Shinya Yamanaka, and Bruce R. Conklin. Stem Cell Reports. 2014 Aug 12;3(2):269-81. PMID: 25254341