Telomerase activity is a biomarker for high grade malignant peripheral nerve sheath tumors in neurofibromatosis type I individuals

Mantripragada, Kiran Kumar ORCID: https://orcid.org/0000-0003-2070-8105, Caley, Matthew, Stephens, Philip ORCID: https://orcid.org/0000-0002-0840-4996, Jones, Christopher John, Kluwe, Lan, Guha, Abhijit Ranjan, Mautner, Victor and Upadhyaya, Meena 2008. Telomerase activity is a biomarker for high grade malignant peripheral nerve sheath tumors in neurofibromatosis type I individuals. Genes Chromosomes and Cancer 47 (3) , pp. 238-246. 10.1002/gcc.20525

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Abstract

Telomerase activity (TA) and the expression of its enzymatic subunits, which have been demonstrated in many tumors, remain poorly investigated in tumors associated with neurofibromatosis type I (NFI). In this study, we analysed the association of TA and the expression of telomerase RNA (TR) and telomerase reverse transcriptase (TERT) in 23 malignant peripheral nerve sheath tumors (MPNST) (17 high grade and 6 low grade tumors), 11 plexiform neurofibromas (PNF) and 6 dermal neurofibromas (DNF). TA was studied using telomerase repeat amplification protocol (TRAP) assay and expression of TR and TERT was investigated using reverse transcription PCR (RT-PCR) and real-time PCR. TA was detected in 14 out of 17 (82%) high grade MPNST, whereas all 6 low grade MPNST and 17 benign tumors were telomerase negative. The TERT transcripts were detected in all high grade MPNST, 50% of the low grade MPNST, and 4 benign tumors. However, the expression level of the TERT strikingly correlated with TA and high grade MPNST. Thus, while TERT expression was similar in both low grade MPNST and PNF (P = 0.115), it was significantly higher in high grade MPNST when compared to either low grade MPNST (P = 0.042), PNF (P = 0.001) or DNF tumors (P = 0.010). These findings indicate that TA and expression level of TERT are potential markers for high grade malignancy in NFI patients. © 2007 Wiley-Liss, Inc.

Item Type:	Article
Status:	Published
Schools:	Dentistry Medicine MRC Centre for Neuropsychiatric Genetics and Genomics (CNGG)
Subjects:	R Medicine > R Medicine (General) R Medicine > RC Internal medicine
Publisher:	Wiley & Sons
ISSN:	1098-2264
Last Modified:	06 Nov 2024 22:23
URI:	https://orca.cardiff.ac.uk/id/eprint/15960

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