Telomeres: protecting chromosomes against genome instability

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Abstract
Telomeric proteins control telomere length and telomere integrity. The six bona fide telomeric binding proteins form shelterin, a complex that maintains chromosome end integrity. Telomere dysfunction can be caused by loss of telomeric repeats or by loss of protective features, both of which are essential for telomere function. Functional telomeres interact with the DNA damage machinery, but the machinery is prevented from processing these ends. Dysfunctional telomeres are recognized as damage and repaired. Repair of dysfunctional telomeres by fusion propels cells into breakage–fusion–bridge cycles, resulting in unequal distribution of genetic material into daughter cells and, therefore, genome instability. Telomere dysfunction and the failure to maintain telomere length is emerging as being the cause of several diseases.