QKI is a critical pre-mRNA alternative splicing regulator of cardiac myofibrillogenesis and contractile function.

ABSTRACT: The RNA-binding protein QKI belongs to the hnRNP K-homology domain protein family, a well-known regulator of pre-mRNA alternative splicing and is associated with several neurodevelopmental disorders. Qki is found highly expressed in developing and adult hearts. By employing the human embryonic stem cell (hESC) to cardiomyocyte differentiation system and generating QKI-deficient hESCs (hESCs-QKI^del) using CRISPR/Cas9 gene editing technology, we analyze the physiological role of QKI in cardiomyocyte differentiation, maturation, and contractile function. hESCs-QKI^del largely maintain normal pluripotency and normal differentiation potential for the generation of early cardiogenic progenitors, but they fail to transition into functional cardiomyocytes. In this work, by usi

SUBMITTER: Chen X 

PROVIDER: S-EPMC7782589 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature