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D4Z4 Coding Transcripts and FSHD

Award Number: R01AR045203
ORGANIZATION: NATIONAL INSTITUTE OF ARTHRITIS & MUSCULOSKELETAL & SKIN DISEASES
OPDIV: NIH
AWARD CLASS: DISCRETIONARY
AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)

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D4Z4 Coding Transcripts and FSHD - PROJECT SUMMARY Facioscapulohumeral dystrophy (FSHD) is caused by the mis-expression of the embryonic transcription factor DUX4 in skeletal muscle. A major mechanism of DUX4-induced cell toxicity in FSHD muscle is through the transcription of the HSATII pericentromeric repeats and their formation of single and double-stranded intranuclear ribonucleoprotein (RNP) complexes. In this proposal, we will determine the molecular components the RNP complexes and mechanisms of their cell toxicity. The significance of this proposal is that it will establish mechanisms of DUX4-induced pathology in FSHD muscle that will support future therapeutic interventions. The long-term goal of this project is to establish the mechanisms of DUX4-induced pathology in FSHD muscle. The overall hypothesis is that expression of DUX4 in FSHD muscle results in the accumulation of stable RNP complexes that alter the post-transcriptional regulation of other cellular RNAs and modulates the innate immune response pathways to inhibit the normal response to double-stranded RNAs. The specific aims of the proposal are: (Aim 1) Determine the RNAs and the proteins that comprise the intranuclear ribonucleoprotein complexes and their contribution to cell toxicity and genome instability. (Aim 2) Determine whether DUX4 regulates the selective degradation or translation of specific subsets of mRNAs. (Aim 3) Test the hypothesis that interactions between DUX4 and components of the innate immune response pathway represent a conserved function to modulate ISG induction by dsRNAs. Together, these aims will further establish the molecular basis of FSHD pathophysiology and identify new opportunities for future therapies.


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: 2025 ( Subtotal = $514,273 )
2025	2025	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	26	12/18/2024	NON-COMPETING CONTINUATION	$514,273
														Subtotal = $514,273

Issue Date FY: 2024 ( Subtotal = $554,272 )
2024	2024	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	25	11/29/2023	NON-COMPETING CONTINUATION	$514,273
2024	2024	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	25	6/10/2024	NON-COMPETING CONTINUATION	$39,999
														Subtotal = $554,272

Issue Date FY: 2023 ( Subtotal = $571,414 )
2023	2023	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	24	12/22/2022	NON-COMPETING CONTINUATION	$514,273
2023	2023	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	24	4/18/2023	NON-COMPETING CONTINUATION	$57,141
														Subtotal = $571,414

Issue Date FY: 2022 ( Subtotal = $566,945 )
2022	2022	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	004	23	9/7/2022	CHANGE OF GRANTEE / TRAINING INSTITUTION / AWARDING INSTITUTION	$9,614
2022	2022	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	22	7/12/2022	NON-COMPETING CONTINUATION	-$389,665
2022	2022	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	22	12/15/2021	NON-COMPETING CONTINUATION	$514,273
2022	2022	FRED HUTCHINSON CANCER CENTER	1100 FAIRVIEW AVE N	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	002	23	7/13/2022	CHANGE OF GRANTEE / TRAINING INSTITUTION / AWARDING INSTITUTION	$442,337
2022	2022	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	003	22	9/7/2022	NON-COMPETING CONTINUATION	-$9,614
														Subtotal = $566,945

Issue Date FY: 2021 ( Subtotal = $571,414 )
2021	2021	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	002	21	5/25/2021	COMPETING CONTINUATION	$17,850
2021	2021	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	21	1/15/2021	COMPETING CONTINUATION	$571,414
2021	2020	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	20	5/25/2021	NON-COMPETING CONTINUATION	-$17,850
														Subtotal = $571,414

Issue Date FY: 2020 ( Subtotal = $424,626 )
2020	2020	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	20	3/30/2020	NON-COMPETING CONTINUATION	$42,463
2020	2020	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	20	12/20/2019	NON-COMPETING CONTINUATION	$382,163
														Subtotal = $424,626

Issue Date FY: 2019 ( Subtotal = $424,626 )
2019	2019	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	19	12/21/2018	NON-COMPETING CONTINUATION	$424,626
														Subtotal = $424,626

Issue Date FY: 2018 ( Subtotal = $443,211 )
2018	2018	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	18	5/2/2018	NON-COMPETING CONTINUATION	$44,321
2018	2018	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	18	12/22/2017	NON-COMPETING CONTINUATION	$398,890
														Subtotal = $443,211

Issue Date FY: 2017 ( Subtotal = $443,211 )
2017	2017	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	17	12/15/2016	NON-COMPETING CONTINUATION	$398,890
2017	2017	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE N J6-300	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	17	6/13/2017	NON-COMPETING CONTINUATION	$44,321
														Subtotal = $443,211

Issue Date FY: 2016 ( Subtotal = $443,211 )
2016	2016	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	16	2/15/2016	COMPETING CONTINUATION	$443,211
														Subtotal = $443,211

Issue Date FY: 2014 ( Subtotal = $364,613 )
2014	2014	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	15	3/11/2014	NON-COMPETING CONTINUATION	$364,613
														Subtotal = $364,613

Issue Date FY: 2013 ( Subtotal = $353,676 )
2013	2013	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	14	1/29/2013	NON-COMPETING CONTINUATION	$335,062
2013	2013	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	14	7/11/2013	NON-COMPETING CONTINUATION	$18,614
														Subtotal = $353,676

Issue Date FY: 2012 ( Subtotal = $372,520 )
2012	2012	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	13	2/27/2012	NON-COMPETING CONTINUATION	$372,520
														Subtotal = $372,520

Issue Date FY: 2011 ( Subtotal = $372,742 )
2011	2011	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	12	1/14/2011	NON-COMPETING CONTINUATION	$349,446
2011	2011	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	001	12	6/8/2011	NON-COMPETING CONTINUATION	$23,296
														Subtotal = $372,742

Issue Date FY: 2010 ( Subtotal = $396,000 )
2010	2010	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	11	1/29/2010	COMPETING CONTINUATION	$396,000
														Subtotal = $396,000

Issue Date FY: 2007 ( Subtotal = $314,636 )
2007	2007	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	10	6/20/2007	NON-COMPETING CONTINUATION	$314,636
														Subtotal = $314,636

Issue Date FY: 2006 ( Subtotal = $324,173 )
2006	2006	FRED HUTCHINSON CANCER RESEARCH CENTER	1100 FAIRVIEW AVE NO J6-330	SEATTLE	WA	98109	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	9	6/29/2006	NON-COMPETING CONTINUATION	$324,173
														Subtotal = $324,173

Issue Date FY: 2005 ( Subtotal = $360,908 )
2005	2005	FRED HUTCHINSON CANCER RESEARCH CENTER	1124 COLUMBIA STREET	SEATTLE	WA	98104	KING	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	8	7/4/2005	NON-COMPETING CONTINUATION	$360,908
														Subtotal = $360,908

Issue Date FY: 2004 ( Subtotal = $361,053 ) (Continued on the next page)
Grand Total All Awards = $9,922,731

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D4Z4 Coding Transcripts and FSHD

Award Number: R01AR045203

ORGANIZATION: NATIONAL INSTITUTE OF ARTHRITIS & MUSCULOSKELETAL & SKIN DISEASES

OPDIV: NIH

AWARD CLASS: DISCRETIONARY

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

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