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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Detalles Bibliográficos
Autores principales: Kliszczak, Maciej, Moralli, Daniela, Jankowska, Julia D., Bryjka, Paulina, Subha Meem, Lamia, Goncalves, Tomas, Hester, Svenja S., Fischer, Roman, Clynes, David, Green, Catherine M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Cell and Developmental Biology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10277729/
https://www.ncbi.nlm.nih.gov/pubmed/37342232
http://dx.doi.org/10.3389/fcell.2023.1175069

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