Differential requirement of SUFU in tissue development discovered in a hypomorphic mouse model.

ABSTRACT: Suppressor of Fused (SUFU) is an essential negative regulator of the Hedgehog (HH) pathway and involved in GLI transcription factor regulation. Due to early embryonic lethality of Sufu^-/- mice, investigations of SUFU's role later in development are limited to conditional, tissue-specific knockout models. In this study we developed a mouse model (Sufu^{Ex456(fl)/Ex456(fl)}) with hypomorphic features where embryos were viable up to E18.5, although with a spectrum of developmental defects of varying severity, including polydactyly, exencephaly and omphalocele. Development of certain tissues, like the skeleton, was more affected than that of others such as skin, which remained largely normal. Interestingly, no apparent changes in the dorso-ventral patterning of the neural tu

SUBMITTER: Hoelzl MA 

PROVIDER: S-EPMC5569906 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature