Posttranscriptional modulation of TERC by PAPD5 inhibition rescues hematopoietic development in dyskeratosis congenita

Reduced levels of TERC, the telomerase RNA component, cause dyskeratosis congenita (DC) in patients harboring mutations in TERC, PARN, NOP10, NHP2, NAF1, or DKC1. Inhibition of the noncanonical poly(A) polymerase PAPD5, or the exosome RNA degradation complex, partially restores TERC levels in immort...

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Bibliografiske detaljer
Udgivet i:Blood
Main Authors: Fok, Wilson Chun, Shukla, Siddharth, Vessoni, Alexandre Teixeira, Brenner, Kirsten Ann, Parker, Roy, Sturgeon, Christopher M., Batista, Luis Francisco Zirnberger
Format: Artigo
Sprog:Inglês
Udgivet: American Society of Hematology 2019
Fag:
Hematopoiesis and Stem Cells
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6428664/
https://ncbi.nlm.nih.gov/pubmed/30728146
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2018-11-885368
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