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Article

GSK3 inhibition rescues growth and telomere dysfunction in dyskeratosis congenita iPSC-derived type II alveolar epithelial cells.

eLife - 13 May 2022

Fernandez Rafael Jesus, Gardner Zachary J G, Slovik Katherine J, Liberti Derek C, Estep Katrina N, Yang Wenli, Chen Qijun, Santini Garrett T, Perez Javier V, Root Sarah, Bhatia Ranvir, Tobias John W, Babu Apoorva, Morley Michael P, Frank David B, Morrisey Edward E, Lengner Christopher J, Johnson F Brad

Abstract excerpt

Dyskeratosis congenita (DC) is a rare genetic disorder characterized by deficiencies in telomere maintenance leading to very short telomeres and the premature onset of certain age-related diseases, including pulmonary fibrosis (PF). PF is thought to derive from epithelial failure, particularly that of type II alveolar epithelial (AT2) cells, which are highly dependent on Wnt signaling during development and adult...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 35559731
DOI: 10.7554/eLife.64430
PMCID: PMC9200405

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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