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The immunoprofile of odontogenic keratocyst (keratocystic odontogenic tumor) that includes expression of PTCH, SMO, GLI-1 and bcl-2 is similar to ameloblastoma but different from odontogenic cysts.

AbstractBACKGROUND:
The aggressive biological behavior of odontogenic keratocysts (OKCs), unlike that of other odontogenic cysts, has argued for its recent re-classification as a neoplasm, 'keratocystic odontogenic tumor'. Identification of mutations in the PTCH gene in some of the OKCs that were expected to produce truncated proteins, resulting in loss of control of the cell cycle, provided additional support for OKCs having a neoplastic nature.
METHODS:
We investigated the immunohistochemical expression of the sonic hedgehog (SHH) signaling pathway-related proteins, PTCH, smoothened (SMO) and GLI-1, and of the SHH-induced bcl-2 oncoprotein in a series of primary OKC (pOKC), recurrent OKC (rOKC) and nevoid basal cell carcinoma syndrome-associated OKCs (NBCCS-OKCs), and compared them to solid ameloblastomas (SAMs), unicystic ameloblastomas (UAMs), 'orthokeratinized' OKCs (oOKCs), dentigerous cysts (DCs) and radicular cysts (RCs).
RESULTS:
All studied lesions expressed the SHH pathway-related proteins in a similar pattern. The expression of bcl-2 in OKCs (pOKCs and NBCCS-OKCs) and SAMs was significantly higher than in oOKCs, DCs and RCs (P < 0.001).
CONCLUSIONS:
The present results of the immunoprofile of OKCs (that includes the expression of the SHH-related proteins and the SHH-induced bcl-2 oncoprotein) further support the notion of OKC having a neoplastic nature. As OKCs vary considerably in their biologic behavior, it is suggested that the quality and quantity of interactions between the SHH and other cell cycle regulatory pathways are likely to work synergistically to define the individual phenotype and corresponding biological behavior of this lesion.
AuthorsM Vered, O Peleg, S Taicher, A Buchner
JournalJournal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology (J Oral Pathol Med) Vol. 38 Issue 7 Pg. 597-604 (Aug 2009) ISSN: 1600-0714 [Electronic] Denmark
PMID19473442 (Publication Type: Comparative Study, Journal Article)
Chemical References
  • GLI1 protein, human
  • Hedgehog Proteins
  • PTCH1 protein, human
  • Patched Receptors
  • Patched-1 Receptor
  • Proto-Oncogene Proteins c-bcl-2
  • Receptors, Cell Surface
  • Receptors, G-Protein-Coupled
  • SMO protein, human
  • Smoothened Receptor
  • Transcription Factors
  • Zinc Finger Protein GLI1
Topics
  • Ameloblastoma (immunology, metabolism, pathology)
  • Analysis of Variance
  • Basal Cell Nevus Syndrome (immunology, metabolism, pathology)
  • Case-Control Studies
  • Gene Expression Regulation, Neoplastic (physiology)
  • Hedgehog Proteins (metabolism)
  • Humans
  • Immunohistochemistry
  • Jaw Diseases (immunology, metabolism, pathology)
  • Jaw Neoplasms (classification, immunology, metabolism, pathology)
  • Odontogenic Cysts (immunology, metabolism, pathology)
  • Patched Receptors
  • Patched-1 Receptor
  • Proto-Oncogene Proteins c-bcl-2 (genetics, metabolism)
  • Receptors, Cell Surface (genetics, metabolism)
  • Receptors, G-Protein-Coupled (genetics, metabolism)
  • Reference Values
  • Second Messenger Systems (physiology)
  • Signal Transduction (physiology)
  • Smoothened Receptor
  • Transcription Factors (genetics, metabolism)
  • Zinc Finger Protein GLI1

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