The deubiquitinating enzyme Usp14 controls ciliogenesis and Hedgehog signaling.

ABSTRACT: Primary cilia are hair-like organelles that play crucial roles in vertebrate development, organogenesis and when dysfunctional result in pleiotropic human genetic disorders called ciliopathies, characterized by overlapping phenotypes, such as renal and hepatic cysts, skeletal defects, retinal degeneration and central nervous system malformations. Primary cilia act as communication hubs to transfer extracellular signals into intracellular responses and are essential for Hedgehog (Hh) signal transduction in mammals. Despite the renewed interest in this ancient organelle of growing biomedical importance, the molecular mechanisms that trigger cilia formation, extension and ciliary signal transduction are still not fully understood. Here we provide, for the first time, evidence that the deubiqu

SUBMITTER: Massa F 

PROVIDER: S-EPMC6381316 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature