Sonic hedgehog signalling proteins are frequently expressed in retinoblastoma and are associated with aggressive clinicopathological features.

Abstract

AIMS:

This study aimed to examine the expression of Sonic hedgehog (SHH) signalling proteins in retinoblastoma and to evaluate its clinical significance.

METHODS:

Seventy-nine enucleated retinoblastoma tumours were investigated immunohistochemically using antibodies against SHH pathway proteins, such as SHH, glioma-associated oncogene homologue (GLI) 1, GLI2, GLI3 and ABC binding cassette G2 (ABCG2). Western blotting of SHH signalling proteins was performed in two retinoblastoma cell lines.

RESULTS:

SHH was expressed in most retinoblastoma cases (78 of 79, 98.7%), with 21 cases (26.6%) showing strong expression. GLI1 and GLI2 were also frequently expressed: 67 of 78 cases (85.9%) and 71 of 77 cases (92.2%), respectively. GLI3, a transcriptional repressor, was expressed at low levels in 23 of the 78 cases (29.5%). High ABCG2 expression was found in 23 of the 78 cases (29.5%). High expression levels of these proteins in retinoblastoma cell lines were confirmed by western blotting. The expression of SHH was associated with advanced stages, local invasion and metastasis (all p<0.05).

CONCLUSIONS:

SHH signalling molecules were frequently expressed in retinoblastoma tumour cells, and high SHH expression was closely related to an advanced disease status. Our results suggest that the SHH signalling pathway may play a role in the progression of retinoblastoma.

• Authors: Ji-Young Choe, Ji Yun Yun, Yoon Kyung Jeon, Se Hoon Kim, Ho-Kyung Choung, Sohee Oh, Mira Park, Ji Eun Kim
• Journal: Journal of clinical pathology (J Clin Pathol) Vol. 68 Issue 1 Pg. 6-11 (Jan 2015) ISSN: 1472-4146 [Electronic] England
• PMID: 25296932 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Chemical References

• Biomarkers, Tumor
• Hedgehog Proteins

Topics

• Biomarkers, Tumor (analysis)
• Brain Neoplasms (metabolism, pathology)
• Child, Preschool
• Female
• Hedgehog Proteins (metabolism)
• Humans
• Immunohistochemistry
• Infant
• Male
• Retinoblastoma (metabolism, pathology)