| First Author | Nguyên-Trân VT | Year | 2000 |
| Journal | Cell | Volume | 102 |
| Issue | 5 | Pages | 671-82 |
| PubMed ID | 11007485 | Mgi Jnum | J:77375 |
| Mgi Id | MGI:2181501 | Doi | 10.1016/s0092-8674(00)00089-1 |
| Citation | Nguyen-Tran VT, et al. (2000) A novel genetic pathway for sudden cardiac death via defects in the transition between ventricular and conduction system cell lineages. Cell 102(5):671-82 |
| abstractText | HF-1 b, an SP1 -related transcription factor, is preferentially expressed in the cardiac conduction system and ventricular myocytes in the heart. Mice deficient for HF-1 b survive to term and exhibit normal cardiac structure and function but display sudden cardiac death and a complete penetrance of conduction system defects, including spontaneous ventricular tachycardia and a high incidence of AV block. Continuous electrocardiographic recordings clearly documented cardiac arrhythmogenesis as the cause of death. Single-cell analysis revealed an anatomic substrate for arrhythmogenesis, including a decrease and mislocalization of connexins and a marked increase in action potential heterogeneity. Two independent markers reveal defects in the formation of ventricular Purkinje fibers. These studies identify a novel genetic pathway for sudden cardiac death via defects in the transition between ventricular and conduction system cell lineages. |
