Wdpcp regulates cellular proliferation and differentiation in the developing limb via hedgehog signaling.

ABSTRACT:

Background

Mice with a loss of function mutation in Wdpcp were described previously to display severe birth defects in the developing heart, neural tube, and limb buds. Further characterization of the skeletal phenotype of Wdpcp null mice was limited by perinatal lethality.

Results

We utilized Prx1-Cre mice to generate limb bud mesenchyme specific deletion of Wdpcp. These mice recapitulated the appendicular skeletal phenotype of the Wdpcp null mice including polydactyl and limb bud signaling defects. Examination of late stages of limb development demonstrated decreased size of cartilage anlagen, delayed calcification, and abnormal growth plates. Utilizing in vitro assays, we demonstrated that loss of Wdpcp in skeletal progenitors lead to loss of hedgehog signaling responsive

SUBMITTER: Langhans MT 

PROVIDER: S-EPMC8258940 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature