A disrupted compartment boundary underlies abnormal cardiac patterning and congenital heart defects.

ABSTRACT: Failure of septation of the interventricular septum (IVS) is the most common congenital heart defect (CHD), but mechanisms for patterning the IVS are largely unknown. We show that a Tbx5 ⁺ /Mef2cAHF ⁺ progenitor lineage forms a compartment boundary bisecting the IVS. This coordinated population originates at a first- and second heart field interface, subsequently forming a morphogenetic nexus. Ablation of Tbx5 ⁺ /Mef2cAHF ⁺ progenitors cause IVS disorganization, right ventricular hypoplasia and mixing of IVS lineages. Reduced dosage of the CHD transcription factor TBX5 disrupts boundary position and integrity, resulting in ventricular septation defects (VSDs) and patterning defects, including Slit2

SUBMITTER: Kathiriya IS 

PROVIDER: S-EPMC10871243 | biostudies-literature | 2024 Mar

REPOSITORIES: biostudies-literature