| First Author | Zhu XJ | Year | 2018 |
| Journal | FEBS Lett | Volume | 592 |
| Issue | 3 | Pages | 356-368 |
| PubMed ID | 29292497 | Mgi Jnum | J:257510 |
| Mgi Id | MGI:6118026 | Doi | 10.1002/1873-3468.12963 |
| Citation | Zhu XJ, et al. (2018) Disruption of Wnt production in Shh lineage causes bone malformation in mice, mimicking human Malik-Percin-type syndactyly. FEBS Lett 592(3):356-368 |
| abstractText | Here, we show that Shh-Cre-mediated deletion of Wntless, the Wnt cargo protein, in mouse posterior limb mesenchyme causes bone syndactyly of the 3rd and 4th digits, resembling the human Malik-Percin type. The Shh descendants gradiently distributed from digit 5 to posterior half of digit 3 in wild-type limbs, however, they abnormally increased in posterior digit 3 in Wntless(Shh-Cre) . Wntless(Shh-Cre) limbs displayed altered expression of hedgehog pathway genes and impaired noncanonical Wnt signaling activity. We further showed that the anterior limb mesenchymal cells in the Wls(Shh-Cre) served as a source of Wnt5a to reorientate the adjacent Wls-lacking Shh lineage cells to move anteriorly and subsequently led to syndactyly, suggesting that aberrant mesenchymal cell movement/condensation may underlie the pathogenesis of syndactyly. |
