| First Author | Shylo NA | Year | 2020 |
| Journal | Dev Biol | Volume | 460 |
| Issue | 2 | Pages | 187-199 |
| PubMed ID | 31887266 | Mgi Jnum | J:286656 |
| Mgi Id | MGI:6405644 | Doi | 10.1016/j.ydbio.2019.12.014 |
| Citation | Shylo NA, et al. (2020) Loss of ciliary transition zone protein TMEM107 leads to heterotaxy in mice. Dev Biol 460(2):187-199 |
| abstractText | Cilia in most vertebrate left-right organizers are involved in the original break in left-right (L-R) symmetry, however, less is known about their roles in subsequent steps of the cascade - relaying the signaling and maintaining the established asymmetry. Here we describe the L-R patterning cascades in two mutants of a ciliary transition zone protein TMEM107, revealing that near-complete loss of cilia in Tmem107(null) leads to left pulmonary isomerism due to the failure of the midline barrier. Contrary, partially retained cilia in the node and the midline of a hypomorphic Tmem107(schlei) mutant appear sufficient for the formation of the midline barrier and establishment and maintenance of the L-R asymmetry. Despite misregulation of Shh signaling in both mutants, the presence of normal Lefty1 expression and midline barrier formation in Tmem107(schlei) mutants, suggests a requirement for cilia, but not necessarily Shh signaling for Lefty1 expression and midline barrier formation. |
