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PMID: 19883731 Published · ppublish English Journal Article Review

The role of the PAX8/PPARgamma fusion oncogene in the pathogenesis of follicular thyroid cancer.

Molecular and cellular endocrinology ·Vol. 321 ·No. 1 ·2010-05-28 ·Pages 50-6

Eberhardt NL, Grebe SK, McIver B, Reddi HV

Abstract

When identified at early stages, most well-differentiated thyroid cancers are readily treated and yield excellent outcomes. Follicular thyroid cancer (FTC) however, when diagnosed at a late stage, may be very resistant to treatment, and exhibits 10-year survival rates less than 40%. Despite substantial progress in recent years, we still have limited understanding of the molecular and biological interrelationships between the various subtypes of benign and malignant follicular thyroid neoplasms. In contrast to the wealth of information available regarding papillary thyroid carcinoma (PTC), the triggering mechanisms of FTC development and the major underlying genetic alterations leading to follicular thyroid carcinogenesis remain obscure. Recent studies have focused on a chromosomal translocation, t(2;3) (q13;p25), fusing PAX8, a transcription factor that is essential for normal thyroid gland development, with the peroxisome proliferator-activated receptor gamma (PPARgamma), a member of the steroid/thyroid nuclear receptor family. This chromatin rearrangement results in the expression of a PAX8/PPARgamma fusion protein, designated PPFP, whose incidence is relatively common in FTC and may represent an initiating event in the genesis of FTC. Here we review progress on the studies of PPFP that assess its involvement in FTC tumorigenesis.

MeSH Terms
Adenocarcinoma, Follicular/genetics Animals Chromosome Aberrations Humans Oncogene Proteins, Fusion/genetics,metabolism PPAR gamma/genetics,metabolism Paired Box Transcription Factors/genetics,metabolism Thyroid Neoplasms/genetics
Chemicals
Oncogene Proteins, Fusion PAX8-PPARgamma fusion protein, human PPAR gamma Paired Box Transcription Factors
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Eberhardt Norman L
Department of Medicine, Division of Endocrinology, Mayo Clinic & Foundation, Rochester, MN 55905, United States. eberhardt@mayo.edu
Grebe Stefan K G
McIver Bryan
Reddi Honey V
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Article Info
Journal
Molecular and cellular endocrinology
Abbr.
Mol Cell Endocrinol
ISSN
1872-8057
DOI
10.1016/j.mce.2009.10.013
Email
eberhardt@mayo.edu
Published
2010-05-28
Epub
2009-00-31
Pages
50-6
Language
English
Region
Ireland
NLM ID
7500844
PMCID
PMC2849860
Subset
IM
Grants
NCI NIH HHS · R01 CA080117-08 · United States
External Links
PubMed source DOI: 10.1016/j.mce.2009.10.013 PMC Full Text View journal details
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