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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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Detalhes bibliográficos
Publicado no:Blood
Main Authors: Fok, Wilson Chun, Shukla, Siddharth, Vessoni, Alexandre Teixeira, Brenner, Kirsten Ann, Parker, Roy, Sturgeon, Christopher M., Batista, Luis Francisco Zirnberger
Formato: Artigo
Idioma:Inglês
Publicado em: American Society of Hematology 2019
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Acesso em linha: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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