Impaired telomere integrity and rRNA biogenesis in PARN‐deficient patients and knock‐out models

Impaired telomere integrity and rRNA biogenesis in PARN‐deficient patients and knock‐out models

Abstract PARN, poly(A)‐specific ribonuclease, regulates the turnover of mRNAs and the maturation and stabilization of the hTR RNA component of telomerase. Biallelic PARN mutations were associated with Høyeraal–Hreidarsson (HH) syndrome, a rare telomere biology disorder that, because of its severity,...

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Bibliographic Details
Main Authors: Maname Benyelles, Harikleia Episkopou, Marie‐Françoise O'Donohue, Laëtitia Kermasson, Pierre Frange, Florian Poulain, Fatma Burcu Belen, Meltem Polat, Christine Bole‐Feysot, Francina Langa‐Vives, Pierre‐Emmanuel Gleizes, Jean‐Pierre deVillartay, Isabelle Callebaut, Anabelle Decottignies, Patrick Revy
Format: Article
Language:English
Published: Springer Nature 2019-07-01
Series:EMBO Molecular Medicine
Subjects:
Høyeraal–Hreidarsson syndrome
p53
PARN
rRNA
shelterin
Online Access:https://doi.org/10.15252/emmm.201810201

Internet

https://doi.org/10.15252/emmm.201810201

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