Loss of FAM111B protease mutated in hereditary fibrosing poikiloderma negatively regulates telomere length

Hereditary fibrosing poikiloderma (HFP) is a rare human dominant negative disorder caused by mutations in the FAM111B gene that encodes a nuclear trypsin-like serine protease. HFP patients present with symptoms including skin abnormalities, tendon contractures, myopathy and lung fibrosis. We charact...

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Bibliographic Details

Published in:	Frontiers in Cell and Developmental Biology
Main Authors:	Maciej Kliszczak, Daniela Moralli, Julia D. Jankowska, Paulina Bryjka, Lamia Subha Meem, Tomas Goncalves, Svenja S. Hester, Roman Fischer, David Clynes, Catherine M. Green
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2023-06-01
Subjects:	FAM111B POIKTMP HFP protease DNA damage telomeres
Online Access:	https://www.frontiersin.org/articles/10.3389/fcell.2023.1175069/full