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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Publicat a:Blood
Autors principals: Fok, Wilson Chun, Shukla, Siddharth, Vessoni, Alexandre Teixeira, Brenner, Kirsten Ann, Parker, Roy, Sturgeon, Christopher M., Batista, Luis Francisco Zirnberger
Format: Artigo
Idioma:Inglês
Publicat: American Society of Hematology 2019
Matèries:
Hematopoiesis and Stem Cells
Accés en línia: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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