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Telomerase deficiency and cancer susceptibility syndromes.
- Source :
-
Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico [Clin Transl Oncol] 2009 Nov; Vol. 11 (11), pp. 711-4. - Publication Year :
- 2009
-
Abstract
- Telomeres from most eukaryotes are composed of repeats of guanine-rich sequences whose main function is to preserve the end of the chromosomes. Telomeres are synthesised by a reverse transcriptase enzyme, telomerase (TERT), which forms part of a ribonucleoprotein complex containing also a RNA template molecule (TERC) and dyskerin. Exhaustion of telomeres during cell divisions triggers a DNA damage response that induces a senescence phenotype. The DNA damage machinery plays an essential role in maintaining the integrity of the genome and also detecting telomere shortening. However in some syndromes that involved mutations either in the telomerase complex genes or those involved in maintaining DNA secondary structure, such as the recQ helicase WRN, a higher frequency in the development of different types of malignancies is observed. We here describe the biology of some of these diseases, together with the molecular modifications in the telomerase complex genes and the impact of these alterations on the development of particular types of cancer.
- Subjects :
- Animals
DNA Damage
Dyskeratosis Congenita genetics
Exodeoxyribonucleases metabolism
Genomics
Humans
Mutation
RNA metabolism
RecQ Helicases metabolism
Telomerase metabolism
Telomere ultrastructure
Werner Syndrome genetics
Werner Syndrome Helicase
Genetic Predisposition to Disease
Neoplasms genetics
Neoplasms metabolism
Telomerase deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 1699-3055
- Volume :
- 11
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
- Publication Type :
- Academic Journal
- Accession number :
- 19917533
- Full Text :
- https://doi.org/10.1007/s12094-009-0432-9