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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Détails bibliographiques
Publié dans:Blood
Auteurs principaux: Fok, Wilson Chun, Shukla, Siddharth, Vessoni, Alexandre Teixeira, Brenner, Kirsten Ann, Parker, Roy, Sturgeon, Christopher M., Batista, Luis Francisco Zirnberger
Format: Artigo
Langue:Inglês
Publié: American Society of Hematology 2019
Sujets:
Hematopoiesis and Stem Cells
Accès en ligne: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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