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Sonic hedgehog signaling is active in human adrenal cortex development and deregulated in adrenocortical tumors

Débora C Gomes1, Letícia F Leal, Livia M Mermejo

  • 1School of Medicine (D.C.G., L.F.L., L.M.M., C.A.S., C.E.M., L.G.T., S.T., F.R., A.C.M., L.N.R., M.C., S.R.R.A.), Ribeirao Preto Medical School-University of Sao Paulo, 14090-900 Ribeirao Preto, Brazil; School of Medicine (M.C.B.V.F., A.C.L.), University of Sao Paulo, 01246-903 Sao Paulo, Brazil; and Boldrini Children's Center (J.A.Y., S.R.B., I.A.C., M.J.M.), 13083-210 Campinas, Brazil.

The Journal of Clinical Endocrinology and Metabolism
|April 10, 2014
PubMed
Abstract

Insights

The Sonic Hedgehog (SHH) pathway is active in human adrenal development and deregulated in adrenocortical tumors (ACTs). SHH pathway inhibition reduced tumor cell viability, suggesting it as a potential therapeutic target.

Area of Science:

  • Endocrinology
  • Developmental Biology
  • Oncology

Background:

  • The Sonic Hedgehog (SHH) pathway is crucial for adrenal cortex development in rodents and implicated in human tumorigenesis.
  • Limited data exists on SHH pathway involvement in human adrenal gland development and cancer.

Purpose of the Study:

  • To investigate the role of the SHH pathway in human adrenal development and adrenocortical tumors (ACTs).
  • To assess the impact of SHH pathway inhibition on an ACT cell line.

Main Methods:

  • Immunohistochemistry and quantitative PCR were used to analyze SHH pathway component expression in normal adrenal glands and ACTs (pediatric and adult).
  • The NCI-H295A ACT cell line was treated with cyclopamine to evaluate SHH inhibition effects on gene expression and cell viability.

Main Results:

  • SHH pathway proteins exhibited specific spatiotemporal expression patterns during human adrenal development.
  • Adult adrenocortical carcinomas showed increased expression of PTCH1, SMO, GLI3, and SUFU compared to normal adult adrenals.
  • Pediatric ACTs displayed decreased mRNA expression of SHH, PTCH1, SMO, GLI1, and GLI3 compared to normal pediatric adrenals.
  • Cyclopamine treatment reduced GLI3, SFRP1, and CTNNB1 mRNA expression, β-catenin staining, and cell viability in vitro.

Conclusions:

  • The SHH pathway is active in human adrenal development and shows differential regulation in ACTs (up-regulated in adult, down-regulated in pediatric).
  • SHH pathway antagonism demonstrated anti-proliferative effects on ACT cells.
  • The SHH pathway is a deregulated pathway in ACTs and represents a potential therapeutic target.

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