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Derivation of lung epithelia from iPS cells for advanced disease modeling

Award Number: R01HL095993
ORGANIZATION: NATIONAL HEART, LUNG, & BLOOD INSTITUTE
OPDIV: NIH
AWARD CLASS: DISCRETIONARY
AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)

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Project Summary/Abstract Diseases affecting the lung parenchyma or alveoli cause significant morbidity and mortality in the US today. Treatments for these diseases are often ineffective and the development of novel therapeutics is hampered by an incomplete understanding of disease pathogenesis due in part to an inability to access living alveolar cells from patients. The derivation of an inexhaustible supply of patient-specific alveolar epithelial cells would allow studies of disease pathogenesis in primary cells carrying each patient’s entire genetic background, and consequently the development of new therapeutics. This application proposes to renew a long-standing project focused on the in vitro derivation of lung epithelial lineages from patient-specific induced pluripotent stem cells (iPSCs). In this renewal we generate an inexhaustible source of functionally mature alveolar epithelial type 2 cells (AEC2s) from iPSCs that we have engineered by reprogramming patient blood or skin specimens. Employing iPSCs generated from children and adults with interstitial lung diseases that arise from heterozygous mutations in surfactant genes, we propose to model the onset of a currently untreatable parenchymal lung disease at its inception, an event that likely begins with AEC2 dysfunction initiated by toxic-gain-of function effects of misfolded proteins. Through three aims we test the hypothesis that AEC2s generated from patient specific iPSCs carrying surfactant pathway mutations exhibit surfactant protein mistrafficking which leads to disrupted proteostasis, a late block in autophagy, broad alveolar progenitor dysfunction, and inflammatory activation.


Issue Date FY	Funding FY	Legal Entity Name	Legal Entity Address	Legal Entity City	Legal Entity State	Legal Entity Zip Code	Legal Entity COUNTY	Legal Entity COUNTRY	Assistance Listing	Award Code	Budget Year	Action Date	Action Type	Action Amount

Issue Date FY: 2022 ( Subtotal = $459,011 )
2022	2022	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	000	13	6/3/2022	NON-COMPETING CONTINUATION	$459,011
														Subtotal = $459,011

Issue Date FY: 2021 ( Subtotal = $465,150 )
2021	2021	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	000	12	5/24/2021	NON-COMPETING CONTINUATION	$465,150
														Subtotal = $465,150

Issue Date FY: 2020 ( Subtotal = $883,614 )
2020	2020	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	000	11	6/10/2020	NON-COMPETING CONTINUATION	$471,114
2020	2020	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Family Smoking Prevention and Tobacco Control Act Regulatory Research	001	11	7/17/2020	SUPPLEMENT FOR EXPANSION	$412,500
														Subtotal = $883,614

Issue Date FY: 2019 ( Subtotal = $493,152 )
2019	2019	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	002	10	9/18/2019	COMPETING CONTINUATION	$5,415
2019	2019	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	000	10	4/26/2019	COMPETING CONTINUATION	$493,152
2019	2017	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	001	9	9/18/2019	NON-COMPETING CONTINUATION	-$5,415
														Subtotal = $493,152

Issue Date FY: 2017 ( Subtotal = $529,979 )
2017	2017	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	008	9	6/2/2017	NON-COMPETING CONTINUATION	$529,979
2017	2016	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	005	8	12/23/2016	EXTENSION WITH OR WITHOUT FUNDS	$3,306
2017	2016	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	007	8	12/30/2016	EXTENSION WITH OR WITHOUT FUNDS	-$3,306
2017	2016	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	004	8	12/23/2016	EXTENSION WITH OR WITHOUT FUNDS	$3,306
2017	2016	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	001	8	11/2/2016	EXTENSION WITH OR WITHOUT FUNDS	$261
2017	2015	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	003	7	12/23/2016	NON-COMPETING CONTINUATION	-$3,306
2017	2015	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	000	7	11/2/2016	NON-COMPETING CONTINUATION	-$261
2017	2015	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	006	7	12/30/2016	NON-COMPETING CONTINUATION	$3,306
2017	2012	TRUSTEES OF BOSTON UNIVERSITY	85 E NEWTON ST M-921	BOSTON	MA	02118	SUFFOLK	USA	Lung Diseases Research	002	4	12/23/2016	NON-COMPETING CONTINUATION	-$3,306
														Subtotal = $529,979

Issue Date FY: 2016 ( Subtotal = $529,979 )
2016	2016	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	8	5/26/2016	EXTENSION WITH OR WITHOUT FUNDS	$529,979
														Subtotal = $529,979

Issue Date FY: 2015 ( Subtotal = $522,028 )
2015	2015	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	7	5/13/2015	NON-COMPETING CONTINUATION	$522,028
														Subtotal = $522,028

Issue Date FY: 2014 ( Subtotal = $521,794 )
2014	2014	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	001	6	6/10/2014	SUPPLEMENT FOR EXPANSION	$120,729
2014	2014	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	6	5/26/2014	NON-COMPETING CONTINUATION	$401,065
														Subtotal = $521,794

Issue Date FY: 2013 ( Subtotal = $389,606 )
2013	2013	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	5	7/18/2013	COMPETING CONTINUATION	$389,606
2013	2012	BOSTON UNIVERSITY	25 BUICK STREET	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	001	4	7/18/2013	NON-COMPETING CONTINUATION	$0
														Subtotal = $389,606

Issue Date FY: 2012 ( Subtotal = $402,188 )
2012	2012	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	4	7/18/2012	NON-COMPETING CONTINUATION	$402,188
														Subtotal = $402,188

Issue Date FY: 2011 ( Subtotal = $406,250 )
2011	2011	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	3	7/19/2011	NON-COMPETING CONTINUATION	$406,250
														Subtotal = $406,250

Issue Date FY: 2010 ( Subtotal = $406,250 )
2010	2010	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	2	7/15/2010	NON-COMPETING CONTINUATION	$406,250
														Subtotal = $406,250

Issue Date FY: 2009 ( Subtotal = $406,250 )
2009	2009	BOSTON UNIVERSITY	25 BUICK ST	BOSTON	MA	02215	SUFFOLK	USA	Lung Diseases Research	000	1	7/20/2009	NEW	$406,250
														Subtotal = $406,250

Grand Total All Awards = $6,415,251

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Derivation of lung epithelia from iPS cells for advanced disease modeling

Award Number: R01HL095993

ORGANIZATION: NATIONAL HEART, LUNG, & BLOOD INSTITUTE

OPDIV: NIH

AWARD CLASS: DISCRETIONARY

AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)

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