Generation of an induced pluripotent stem cell line (SHETi004-A) from a Chinese Han child with left bundle branch block.

ABSTRACT

Desmin (DES) is an important intermediate filament protein associated with the extrasarcomeric cytoskeleton and cellular function that was first reported to be associated with cardiac conduction disease and cardiomyopathy in 1998. We generated an induced pluripotent stem cell (iPSC) line from the left bundle branch block (LBBB) patient's peripheral blood mononuclear cells using Sendai virus-mediated reprogramming. The iPSCs exhibited stable amplification, expressed pluripotent markers, and spontaneously differentiated into three layers in vitro. Additionally, it showed a normal diploid karyotype and maintained the pathogenic mutation in DES. Hence, the iPSC line provided a platform for exploring LBBB mechanisms associated with DES mutations.