Telomerase RNA levels limit the telomere length equilibrium

CW Greider - Cold Spring Harbor symposia on quantitative …, 2006 - symposium.cshlp.org
Cold Spring Harbor symposia on quantitative biology, 2006symposium.cshlp.org
Small functional RNAs play essential roles in many biological processes. Regulating the
level of these small RNAs can beas important as maintaining their function in cells. The
telomerase RNA is maintained in cells at a steady-state level wheresmall changes in
concentration can have a profound impact on function. Cells that have half the level of the
telomerase RNAcannot maintain telomeres through many cell divisions. People who are
heterozygous for telomerase RNA mutations have thediseases dyskeratosis congenita and …
Abstract
Small functional RNAs play essential roles in many biological processes. Regulating the level of these small RNAs can beas important as maintaining their function in cells. The telomerase RNA is maintained in cells at a steady-state level wheresmall changes in concentration can have a profound impact on function. Cells that have half the level of the telomerase RNAcannot maintain telomeres through many cell divisions. People who are heterozygous for telomerase RNA mutations have thediseases dyskeratosis congenita and aplastic anemia, caused by short telomeres that result in loss of tissue renewal capacity. Mice heterozygous for telomerase RNA show haploinsufficiency in telomere length maintenance and also show loss of tissuerenewal capacity. It is remarkable that small changes in the level of this functional RNA can have such profound effects incells. This tight regulation highlights the importance of controlling the action of telomerase in cells.
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