Ligand-dependent Hedgehog pathway activation in Rhabdomyosarcoma: the oncogenic role of the ligands.

ABSTRACT:

Background

Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children. The Hedgehog (HH) pathway is known to develop an oncogenic role in RMS. However, the molecular mechanism that drives activation of the pathway in RMS is not well understood.

Methods

The expression of HH ligands was studied by qPCR, western blot and immunohistochemistry. Functional and animal model studies were carried out with cells transduced with shRNAs against HH ligands or treated with HH-specific inhibitors (Vismodegib and MEDI-5304). Finally, the molecular characterisation of an off-target effect of Vismodegib was also made.

Results

The results showed a prominent expression of HH ligands supporting an autocrine ligand-dependent activation of the pathway. A comparison of

SUBMITTER: Almazan-Moga A 

PROVIDER: S-EPMC5672936 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature