[HTML][HTML] Molecular pathogenesis of pulmonary fibrosis, with focus on pathways related to TGF-β and the ubiquitin-proteasome pathway

N Inui, S Sakai, M Kitagawa - International journal of molecular sciences, 2021 - mdpi.com
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease. During
the past decade, novel pathogenic mechanisms of IPF have been elucidated that have …

Molecular Mechanism of Pancreatic and Salivary Gland Fluid and HCO3 Secretion

MG Lee, E Ohana, HW Park, D Yang… - Physiological …, 2012 - journals.physiology.org
Fluid and HCO3− secretion is a vital function of all epithelia and is required for the survival of
the tissue. Aberrant fluid and HCO3− secretion is associated with many epithelial diseases …

[HTML][HTML] Mammalian HECT ubiquitin-protein ligases: biological and pathophysiological aspects

M Scheffner, S Kumar - Biochimica et Biophysica Acta (BBA)-Molecular …, 2014 - Elsevier
Members of the HECT family of E3 ubiquitin-protein ligases are characterized by a C-
terminal HECT domain that catalyzes the covalent attachment of ubiquitin to substrate …

System-wide modulation of HECT E3 ligases with selective ubiquitin variant probes

W Zhang, KP Wu, MA Sartori, HB Kamadurai… - Molecular cell, 2016 - cell.com
HECT-family E3 ligases ubiquitinate protein substrates to control virtually every eukaryotic
process and are misregulated in numerous diseases. Nonetheless, understanding of HECT …

The development and plasticity of alveolar type 1 cells

J Yang, BJ Hernandez, D Martinez Alanis… - …, 2016 - journals.biologists.com
Alveolar type 1 (AT1) cells cover> 95% of the gas exchange surface and are extremely thin
to facilitate passive gas diffusion. The development of these highly specialized cells and its …

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

AL Garland, WG Walton, RD Coakley, CD Tan… - Proceedings of the …, 2013 - pnas.org
The ability to maintain proper airway surface liquid (ASL) volume homeostasis is vital for
mucus hydration and clearance, which are essential aspects of the mammalian lung's innate …

NEDD4-2 (NEDD4L): the ubiquitin ligase for multiple membrane proteins

P Goel, JA Manning, S Kumar - Gene, 2015 - Elsevier
Abstract NEDD4-2 (also known as NEDD4L, neural precursor cell expressed
developmentally down-regulated 4-like) is a ubiquitin protein ligase of the Nedd4 family …

Conditional deletion of Nedd4-2 in lung epithelial cells causes progressive pulmonary fibrosis in adult mice

J Duerr, DHW Leitz, M Szczygiel, D Dvornikov… - Nature …, 2020 - nature.com
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease
characterized by patchy scarring of the distal lung with limited therapeutic options and poor …

The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

PJ Moore, R Tarran - Expert opinion on therapeutic targets, 2018 - Taylor & Francis
Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

TGF-β directs trafficking of the epithelial sodium channel ENaC which has implications for ion and fluid transport in acute lung injury

DM Peters, I Vadász, Ł Wujak, M Wygrecka… - Proceedings of the …, 2014 - pnas.org
TGF-β is a pathogenic factor in patients with acute respiratory distress syndrome (ARDS), a
condition characterized by alveolar edema. A unique TGF-β pathway is described, which …