Faculty

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Amy L. Firth, PhD
Assistant Professor of Medicine
Medicine
IRD 720 2020 Zonal Avenue Off Campus Los Angeles
+1 323 442 1833

Overview

Many of us take our lungs for granted yet they perform a function that is critical for life, respiration. Unfortunately millions of Americans suffer from one of a number of debilitating lung diseases making the subconscious action of breathing a very conscious and difficult activity. Studying human lung disease and, in particular, the way in which a lung disease develops in humans is exceptionally difficult in a dish. For me this was the challenge that became the focus of my postdoctoral research at The Salk Institute for Biological Studies – to create a reproducible and effective way to study human lung development and disease in a dish.

My laboratory will focus on utilizing patient derived induced pluripotent stem cells (iPSC) and state-of-the-art gene editing technologies, such as CRISPR, to a) model and study mechanisms of lung disease development and b) investigate approaches for tissue regeneration and stem cell mediated repair of the airway epithelium.

Firth Lab Website

Awards

House of Commons: Finalist, Britain's Top Young Researcher in Biosciences and Medicine, 2005

American Heart Association : Finalist, Cournand and Comroe Young Investigator Award, 2009

American Physiology Society: Caroline tum Suden/Francis A. Hellebrandt Professional Opportunity Award, 2014

Publications

Kim HW, Li H, Kim HS, Shin SE, Jung WK, Ha KS, Han ET, Hong SH, Choi IW, Firth AL, Bang H, Park WS. The anti-diabetic drug repaglinide induces vasorelaxation via activation of PKA and PKG in aortic smooth muscle. Vascul Pharmacol. 2016 Sep; 84:38-46. View in: PubMed

Hong da H, Li H, Kim HW, Kim HS, Son YK, Yang SR, Park JR, Ha KS, Han ET, Hong SH, Firth AL, Na SH, Park WS. Alterations of voltage-dependent K(+) channels in the mesenteric artery during the early and chronic phases of diabetes. Clin Exp Pharmacol Physiol. 2016 Sep; 43(9):808-17. View in: PubMed

Li H, Kim HS, Kim HW, Shin SE, Jung WK, Ha KS, Han ET, Hong SH, Firth AL, Bae YM, Choi IW, Park WS. The class III anti-arrhythmic agent, amiodarone, inhibits voltage-dependent K(+) channels in rabbit coronary arterial smooth muscle cells. Naunyn Schmiedebergs Arch Pharmacol. 2016 Jul; 389(7):713-21. View in: PubMed

Firth AL, Menon T, Parker GS, Qualls SJ, Lewis BM, Ke E, Dargitz CT, Wright R, Khanna A, Gage FH, Verma IM. Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs. Cell Rep. 2015 Sep 1; 12(9):1385-90. View in: PubMed

Menon T, Firth AL, Scripture-Adams DD, Galic Z, Qualls SJ, Gilmore WB, Ke E, Singer O, Anderson LS, Bornzin AR, Alexander IE, Zack JA, Verma IM. Lymphoid Regeneration from Gene-Corrected SCID-X1 Subject-Derived iPSCs. Cell Stem Cell. 2015 Apr 2; 16(4):367-72. View in: PubMed

Li H, Choi IW, Hong da H, Son YK, Na SH, Jung WK, Firth AL, Jung ID, Park YM, Park WS. W-7 inhibits voltage-dependent K(+) channels independent of calmodulin activity in rabbit coronary arterial smooth muscle cells. Eur J Pharmacol. 2015 Mar 5; 750C:14-9. View in: PubMed

Hong da H, Li H, Kim HS, Kim HW, Shin SE, Jung WK, Na SH, Choi IW, Firth AL, Park WS, Kim DJ. The Effects of the Selective Serotonin Reuptake Inhibitor Fluvoxamine on Voltage-Dependent K(+) Channels in Rabbit Coronary Arterial Smooth Muscle Cells. Biol Pharm Bull. 2015; 38(8):1208-13. View in: PubMed

Firth AL, Dargitz CT, Qualls SJ, Menon T, Wright R, Singer O, Gage FH, Khanna A, Verma IM. Generation of multiciliated cells in functional airway epithelia from human induced pluripotent stem cells. Proc Natl Acad Sci U S A. 2014 Apr 29; 111(17):E1723-30. View in: PubMed

Ogawa A, Firth AL, Ariyasu S, Yamadori I, Matsubara H, Song S, Fraidenburg DR, Yuan JX. Thrombin-mediated activation of Akt signaling contributes to pulmonary vascular remodeling in pulmonary hypertension. Physiol Rep. 2013 Dec 1; 1(7):e00190. View in: PubMed

Son YK, Park H, Firth AL, Park WS. Side-effects of protein kinase inhibitors on ion channels. J Biosci. 2013 Dec; 38(5):937-49. View in: PubMed

Firth AL, Yuan JX. Human models for smooth muscle cell differentiation. Focus on "A novel in vitro model system for smooth muscle differentiation from human embryonic stem cell-derived mesenchymal cells". Am J Physiol Cell Physiol. 2013 Feb 15; 304(4):C287-8. View in: PubMed

Firth AL, Won JY, Park WS. Regulation of ca(2+) signaling in pulmonary hypertension. Korean J Physiol Pharmacol. 2013 Feb; 17(1):1-8. View in: PubMed

Firth AL, Choi IW, Park WS. Animal models of pulmonary hypertension: Rho kinase inhibition. Prog Biophys Mol Biol. 2012 Aug; 109(3):67-75. View in: PubMed

Firth AL, Yuan JX. "Ether-à-go-go" proliferation of iPSC-derived mesenchymal stem cells. Focus on "Regulation of cell proliferation of human induced pluripotent stem cell-derived mesenchymal stem cells via ether-à-go-go 1 (hEAG1) potassium channel". Am J Physiol Cell Physiol. 2012 Jul 15; 303(2):C113-4. View in: PubMed

Macfarlan TS, Gifford WD, Driscoll S, Lettieri K, Rowe HM, Bonanomi D, Firth A, Singer O, Trono D, Pfaff SL. Embryonic stem cell potency fluctuates with endogenous retrovirus activity. Nature. 2012 Jul 5; 487(7405):57-63. View in: PubMed

Ogawa A, Firth AL, Smith KA, Maliakal MV, Yuan JX. PDGF enhances store-operated Ca2+ entry by upregulating STIM1/Orai1 via activation of Akt/mTOR in human pulmonary arterial smooth muscle cells. Am J Physiol Cell Physiol. 2012 Jan 15; 302(2):C405-11. View in: PubMed

Firth AL, Yuan JX. Identification of functional progenitor cells in the pulmonary vasculature. Pulm Circ. 2012 Jan-Mar; 2(1):84-100. View in: PubMed

Son YK, Hong da H, Kim DJ, Firth AL, Park WS. Direct effect of protein kinase C inhibitors on cardiovascular ion channels. BMB Rep. 2011 Sep; 44(9):559-65. View in: PubMed

Makino A, Firth AL, Yuan JX. Endothelial and smooth muscle cell ion channels in pulmonary vasoconstriction and vascular remodeling. Compr Physiol. 2011 Jul; 1(3):1555-602. View in: PubMed

Woods NB, Parker AS, Moraghebi R, Lutz MK, Firth AL, Brennand KJ, Berggren WT, Raya A, Izpisúa Belmonte JC, Gage FH, Verma IM. Brief report: efficient generation of hematopoietic precursors and progenitors from human pluripotent stem cell lines. Stem Cells. 2011 Jul; 29(7):1158-64. View in: PubMed

Tang T, Hammond HK, Firth A, Yang Y, Gao MH, Yuan JX, Lai NC. Adenylyl cyclase 6 improves calcium uptake and left ventricular function in aged hearts. J Am Coll Cardiol. 2011 May 3; 57(18):1846-55. View in: PubMed

Gao MH, Tang T, Lai NC, Miyanohara A, Guo T, Tang R, Firth AL, Yuan JX, Hammond HK. Beneficial effects of adenylyl cyclase type 6 (AC6) expression persist using a catalytically inactive AC6 mutant. Mol Pharmacol. 2011 Mar; 79(3):381-8. View in: PubMed

Sugano Y, Lai NC, Gao MH, Firth AL, Yuan JX, Lew WY, Hammond HK. Activated expression of cardiac adenylyl cyclase 6 reduces dilation and dysfunction of the pressure-overloaded heart. Biochem Biophys Res Commun. 2011 Feb 18; 405(3):349-55. View in: PubMed

Ko EA, Park WS, Firth AL, Hong da H, Choi SW, Heo HJ, Kim MH, Noh JH, Ko JH, Kim N, Earm YE, Song DK, Han J. Increased sensitivity of serotonin on the voltage-dependent K+ channels in mesenteric arterial smooth muscle cells of OLETF rats. Prog Biophys Mol Biol. 2010 Sep; 103(1):88-94. View in: PubMed

Ko EA, Park WS, Firth AL, Kim N, Yuan JX, Han J. Pathophysiology of voltage-gated K+ channels in vascular smooth muscle cells: modulation by protein kinases. Prog Biophys Mol Biol. 2010 Sep; 103(1):95-101. View in: PubMed

Firth AL, Mandel J, Yuan JX. Idiopathic pulmonary arterial hypertension. Dis Model Mech. 2010 May-Jun; 3(5-6):268-73. View in: PubMed

Firth AL, Yao W, Ogawa A, Madani MM, Lin GY, Yuan JX. Multipotent mesenchymal progenitor cells are present in endarterectomized tissues from patients with chronic thromboembolic pulmonary hypertension. Am J Physiol Cell Physiol. 2010 May; 298(5):C1217-25. View in: PubMed

Firth AL, Yao W, Remillard CV, Ogawa A, Yuan JX. Upregulation of Oct-4 isoforms in pulmonary artery smooth muscle cells from patients with pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2010 Apr; 298(4):L548-57. View in: PubMed

Park WS, Firth AL, Han J, Ko EA. Patho-, physiological roles of voltage-dependent K+ channels in pulmonary arterial smooth muscle cells. J Smooth Muscle Res. 2010; 46(2):89-105. View in: PubMed

Ogawa A, Firth AL, Yao W, Madani MM, Kerr KM, Auger WR, Jamieson SW, Thistlethwaite PA, Yuan JX. Inhibition of mTOR attenuates store-operated Ca2+ entry in cells from endarterectomized tissues of patients with chronic thromboembolic pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol. 2009 Oct; 297(4):L666-76. View in: PubMed

Firth AL, Platoshyn O, Brevnova EE, Burg ED, Powell F, Haddad GH, Yuan JX. Hypoxia selectively inhibits KCNA5 channels in pulmonary artery smooth muscle cells. Ann N Y Acad Sci. 2009 Oct; 1177:101-11. View in: PubMed

Yao W, Firth AL, Sacks RS, Ogawa A, Auger WR, Fedullo PF, Madani MM, Lin GY, Sakakibara N, Thistlethwaite PA, Jamieson SW, Rubin LJ, Yuan JX. Identification of putative endothelial progenitor cells (CD34+CD133+Flk-1+) in endarterectomized tissue of patients with chronic thromboembolic pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol. 2009 Jun; 296(6):L870-8. View in: PubMed

Firth AL, Yau J, White A, Chiles PG, Marsh JJ, Morris TA, Yuan JX. Chronic exposure to fibrin and fibrinogen differentially regulates intracellular Ca2+ in human pulmonary arterial smooth muscle and endothelial cells. Am J Physiol Lung Cell Mol Physiol. 2009 Jun; 296(6):L979-86. View in: PubMed

Firth AL, Gordienko DV, Yuill KH, Smirnov SV. Cellular localization of mitochondria contributes to Kv channel-mediated regulation of cellular excitability in pulmonary but not mesenteric circulation. Am J Physiol Lung Cell Mol Physiol. 2009 Mar; 296(3):L347-60. View in: PubMed

Firth AL. Fine tuning I(CRAC): the interactions of STIM-1 and Orai. J Physiol. 2009 Jan 15; 587(Pt 1):15-6. View in: PubMed

Firth AL, Yuan JX. Bringing down the ROS: a new therapeutic approach for PPHN. Am J Physiol Lung Cell Mol Physiol. 2008 Dec; 295(6):L976-8. View in: PubMed

Sacks RS, Firth AL, Remillard CV, Agange N, Yau J, Ko EA, Yuan JX. Thrombin-mediated increases in cytosolic [Ca2+] involve different mechanisms in human pulmonary artery smooth muscle and endothelial cells. Am J Physiol Lung Cell Mol Physiol. 2008 Dec; 295(6):L1048-55. View in: PubMed

Ogawa A, Firth AL, Yao W, Rubin LJ, Yuan JX. Prednisolone inhibits PDGF-induced nuclear translocation of NF-kappaB in human pulmonary artery smooth muscle cells. Am J Physiol Lung Cell Mol Physiol. 2008 Oct; 295(4):L648-57. View in: PubMed

Firth AL, Yuill KH, Smirnov SV. Mitochondria-dependent regulation of Kv currents in rat pulmonary artery smooth muscle cells. Am J Physiol Lung Cell Mol Physiol. 2008 Jul; 295(1):L61-70. View in: PubMed

Schach C, Firth AL, Xu M, Remillard CV, Patel HH, Insel PA, Yuan JX. Regulation of pulmonary vasoconstriction by agonists and caveolae. Exp Lung Res. 2008 May; 34(4):195-208. View in: PubMed

Tang T, Gao MH, Lai NC, Firth AL, Takahashi T, Guo T, Yuan JX, Roth DM, Hammond HK. Adenylyl cyclase type 6 deletion decreases left ventricular function via impaired calcium handling. Circulation. 2008 Jan 1; 117(1):61-9. View in: PubMed

Wu W, Platoshyn O, Firth AL, Yuan JX. Hypoxia divergently regulates production of reactive oxygen species in human pulmonary and coronary artery smooth muscle cells. Am J Physiol Lung Cell Mol Physiol. 2007 Oct; 293(4):L952-9. View in: PubMed

Ko EA, Burg ED, Platoshyn O, Msefya J, Firth AL, Yuan JX. Functional characterization of voltage-gated K+ channels in mouse pulmonary artery smooth muscle cells. Am J Physiol Cell Physiol. 2007 Sep; 293(3):C928-37. View in: PubMed

Firth AL, Remillard CV, Yuan JX. TRP channels in hypertension. Biochim Biophys Acta. 2007 Aug; 1772(8):895-906. View in: PubMed

Tammaro P, Smith AL, Crowley BL, Smirnov SV. Modulation of the voltage-dependent K+ current by intracellular Mg2+ in rat aortic smooth muscle cells. Cardiovasc Res. 2005 Feb 1; 65(2):387-96. View in: PubMed

Tammaro P, Smith AL, Hutchings SR, Smirnov SV. Pharmacological evidence for a key role of voltage-gated K+ channels in the function of rat aortic smooth muscle cells. Br J Pharmacol. 2004 Sep; 143(2):303-17. View in: PubMed

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