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A regulatory loop connecting WNT signaling and telomere capping: possible therapeutic implications for dyskeratosis congenita
- Source :
- Annals of the New York Academy of Sciences. 1418:56-68
- Publication Year :
- 2018
- Publisher :
- Wiley, 2018.
-
Abstract
- The consequences of telomere dysfunction are most apparent in rare inherited syndromes caused by genetic deficiencies in factors that normally maintain telomeres. The principal disease is known as dyskeratosis congenita (DC), but other syndromes with similar underlying genetic defects share some clinical aspects with this disease. Currently, there are no curative therapies for these diseases of telomere dysfunction. Here, we review recent findings demonstrating that dysfunctional (i.e., uncapped) telomeres can downregulate the WNT pathway, and that restoration of WNT signaling helps to recap telomeres by increasing expression of shelterins, proteins that naturally bind and protect telomeres. We discuss how these findings are different from previous observations connecting WNT and telomere biology, and discuss potential links between WNT and clinical manifestations of the DC spectrum of diseases. Finally, we argue for exploring the use of WNT agonists, specifically lithium, as a possible therapeutic approach for patients with DC.
- Subjects :
- 0301 basic medicine
Telomerase
General Neuroscience
Telomere Capping
Wnt signaling pathway
Disease
Biology
medicine.disease
General Biochemistry, Genetics and Molecular Biology
Telomere
03 medical and health sciences
Therapeutic approach
030104 developmental biology
History and Philosophy of Science
Downregulation and upregulation
medicine
Cancer research
Dyskeratosis congenita
Subjects
Details
- ISSN :
- 00778923
- Volume :
- 1418
- Database :
- OpenAIRE
- Journal :
- Annals of the New York Academy of Sciences
- Accession number :
- edsair.doi...........919d5dc9b41c45400a14d8467b12077e