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Genes 2015, 6(3), 500-511; doi:10.3390/genes6030500

Hedgehog Signaling in Malignant Pleural Mesothelioma

1
University Hospital Zurich, Laboratory of Molecular Oncology, Clinic of Oncology, Haeldeliweg 4, 8044 Zürich, Switzerland
2
University Hospital Zurich, Division of Thoracic Surgery, Raemistrasse 100, 8091 Zurich, Switzerland
*
Author to whom correspondence should be addressed.
Academic Editor: Paul Cahill
Received: 26 May 2015 / Revised: 24 June 2015 / Accepted: 30 June 2015 / Published: 8 July 2015
(This article belongs to the Special Issue Hedgehog Signaling Gene Regulatory Networks)
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Abstract

Malignant pleural mesothelioma (MPM) is a cancer associated with exposure to asbestos fibers, which accumulate in the pleural space, damage tissue and stimulate regeneration. Hedgehog signaling is a pathway important during embryonic mesothelium development and is inactivated in adult mesothelium. The pathway is reactivated in some MPM patients with poor clinical outcome, mainly mediated by the expression of the ligands. Nevertheless, mutations in components of the pathway have been observed in a few cases. Data from different MPM animal models and primary culture suggest that both autocrine and paracrine Hedgehog signaling are important to maintain tumor growth. Drugs inhibiting the pathway at the level of the smoothened receptor (Smo) or glioma-associated protein transcription factors (Gli) have been used mostly in experimental models. For clinical development, biomarkers are necessary for the selection of patients who can benefit from Hedgehog signaling inhibition. View Full-Text
Keywords: Hedgehog signaling; malignant pleural mesothelioma; Gli-1; desert Hedgehog; HHip; TCGA; autocrine signaling; paracrine signaling Hedgehog signaling; malignant pleural mesothelioma; Gli-1; desert Hedgehog; HHip; TCGA; autocrine signaling; paracrine signaling
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Felley-Bosco, E.; Opitz, I.; Meerang, M. Hedgehog Signaling in Malignant Pleural Mesothelioma. Genes 2015, 6, 500-511.

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