Resident interstitial lung fibroblasts and their role in alveolar stem cell niche development, homeostasis, injury, and regeneration

…, M Riccetti, AKT Perl - Stem cells translational …, 2021 - academic.oup.com
Developing, regenerating, and repairing a lung all require interstitial resident fibroblasts (iReFs)
to direct the behavior of the epithelial stem cell niche. During lung development, distal …

Molecular mechanisms controlling lung morphogenesis: Section Editor: Roderick R McInnes e‐mail: mcinnes@ sickkids. on. ca

AKT Perl, JA Whitsett - Clinical genetics, 1999 - Wiley Online Library
The complex process of lung formation is determined by the action of numerous genes that
influence cell commitment, differentiation, and proliferation. This review summarizes current …

Early restriction of peripheral and proximal cell lineages during formation of the lung

AKT Perl, SE Wert, A Nagy, CG Lobe… - Proceedings of the …, 2002 - National Acad Sciences
To establish the timing of lineage restriction among endodermal derivatives, we developed
a method to label permanently subsets of lung precursor cells at defined times during …

Signaling pathways in the epithelial origins of pulmonary fibrosis

WD Hardie, JS Hagood, V Dave, AKT Perl, AKT Perl… - Cell Cycle, 2010 - Taylor & Francis
Pulmonary fibrosis complicates a number of disease processes and leads to substantial
morbidity and mortality. Idiopathic pulmonary fibrosis (IPF) is perhaps the most pernicious and …

[HTML][HTML] Single-cell RNA sequencing identifies diverse roles of epithelial cells in idiopathic pulmonary fibrosis

…, M Guo, J Tang, KA Wikenheiser-Brokamp, AKT Perl… - JCI insight, 2016 - ncbi.nlm.nih.gov
Idiopathic pulmonary fibrosis (IPF) is a lethal interstitial lung disease characterized by airway
remodeling, inflammation, alveolar destruction, and fibrosis. We utilized single-cell RNA …

Conditional gene expression in the respiratory epithelium of the mouse

AKT Perl, JW Tichelaar, JA Whitsett - Transgenic research, 2002 - Springer
Transgenic mouse models mediating conditional temporal and spatial regulation of gene
expression to the respiratory epithelium were developed utilizing the reverse tetracycline …

Role of Sonic hedgehog in patterning of tracheal–bronchial cartilage and the peripheral lung

…, SE Wert, JC Clark, Y Xu, AKT Perl… - … dynamics: an official …, 2004 - Wiley Online Library
Sonic hedgehog (Shh) was conditionally deleted in respiratory epithelial cells of the embryonic
lung in vivo. Deletion of Shh before embryonic day (E) 13.5 resulted in respiratory failure …

FGF-10 disrupts lung morphogenesis and causes pulmonary adenomas in vivo

…, SE Wert, N Itoh, AKT Perl… - … of Physiology-Lung …, 2001 - journals.physiology.org
Transgenic mice in which fibroblast growth factor (FGF)-10 was expressed in the lungs of
fetal and postnatal mice were generated with a doxycycline-inducible system controlled by …

Dynamic regulation of platelet-derived growth factor receptor α expression in alveolar fibroblasts during realveolarization

…, T Acciani, T Le Cras, C Lutzko, AKT Perl - American journal of …, 2012 - atsjournals.org
… Correspondence and requests for reprints should be addressed to Anne-Karina T. Perl,
Ph.D., Children's Hospital Medical Center, Divisions of Neonatology and Pulmonary Biology, …

C/EBPα is required for lung maturation at birth

PC Martis, JA Whitsett, Y Xu, AKT Perl, H Wan… - 2006 - journals.biologists.com
Epithelial cells lining the peripheral lung synthesize pulmonary surfactant that reduces
surface tension at the air-liquid interface. Lack of surfactant lipids and proteins in the lungs …