[HTML][HTML] TRP channels in hypertension

AL Firth, CV Remillard, JXJ Yuan - … Acta (BBA)-Molecular Basis of Disease, 2007 - Elsevier
Pulmonary and systemic arterial hypertension are associated with profound alterations in
Ca2+ homeostasis and smooth muscle cell proliferation. A novel class of non-selective …

Pathophysiology of voltage-gated K+ channels in vascular smooth muscle cells: modulation by protein kinases

EA Ko, WS Park, AL Firth, N Kim, JXJ Yuan… - Progress in biophysics …, 2010 - Elsevier
In this review, the pathological alteration and clinical relevance of voltage-gated K+ (Kv)
channels and their specific regulation by protein kinase-dependent signaling in vascular …

Idiopathic pulmonary arterial hypertension

AL Firth, J Mandel, JXJ Yuan - Disease models & …, 2010 - journals.biologists.com
Despite improved understanding of the pathobiology of pulmonary arterial hypertension
(PAH), it remains a severe and progressive disease, usually culminating in right heart …

[PDF][PDF] Functional gene correction for cystic fibrosis in lung epithelial cells generated from patient iPSCs

AL Firth, T Menon, GS Parker, SJ Qualls, BM Lewis… - Cell reports, 2015 - cell.com
Lung disease is a major cause of death in the United States, with current therapeutic
approaches serving only to manage symptoms. The most common chronic and life …

Generation of multiciliated cells in functional airway epithelia from human induced pluripotent stem cells

AL Firth, CT Dargitz, SJ Qualls… - Proceedings of the …, 2014 - National Acad Sciences
Despite therapeutic advancement, pulmonary disease still remains a major cause of
morbidity and mortality around the world. Opportunities to study human lung disease either …

PDGF enhances store-operated Ca2+ entry by upregulating STIM1/Orai1 via activation of Akt/mTOR in human pulmonary arterial smooth muscle cells

A Ogawa, AL Firth, KA Smith… - American Journal of …, 2012 - journals.physiology.org
Platelet-derived growth factor (PDGF) and its receptor are known to be substantially
elevated in lung tissues and pulmonary arterial smooth muscle cells (PASMC) isolated from …

Grp78 Loss in Epithelial Progenitors Reveals an Age-linked Role for Endoplasmic Reticulum Stress in Pulmonary Fibrosis

Z Borok, M Horie, P Flodby, H Wang, Y Liu… - American Journal of …, 2020 - atsjournals.org
Rationale: Alveolar epithelial cell (AEC) injury and dysregulated repair are implicated in the
pathogenesis of pulmonary fibrosis. Endoplasmic reticulum (ER) stress in AEC has been …

[PDF][PDF] Lymphoid regeneration from gene-corrected SCID-X1 subject-derived iPSCs

T Menon, AL Firth, DD Scripture-Adams, Z Galic… - Cell stem cell, 2015 - cell.com
Summary X-linked Severe Combined Immunodeficiency (SCID-X1) is a genetic disease that
leaves newborns at high risk of serious infection and a predicted life span of less than 1 year …

Adenylyl cyclase type 6 deletion decreases left ventricular function via impaired calcium handling

T Tang, MH Gao, NC Lai, AL Firth, T Takahashi, T Guo… - Circulation, 2008 - Am Heart Assoc
Background—Adenylyl cyclases (ACs) are a family of effector molecules for G-protein–
coupled receptors. The 2 ACs most abundantly expressed in cardiac myocytes are types 5 …

Establishment of the early cilia preassembly protein complex during motile ciliogenesis

A Horani, A Ustione, T Huang, AL Firth… - Proceedings of the …, 2018 - National Acad Sciences
Motile cilia are characterized by dynein motor units, which preassemble in the cytoplasm
before trafficking into the cilia. Proteins required for dynein preassembly were discovered by …