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| Status |
Public on Oct 14, 2011 |
| Title |
SMRT-RID mutation linked to Type I Pneumocyte Associated Respiratory Distress Syndrome via TR repression |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| Summary |
We show that knock-in mutations of the nuclear corepressor SMRT in C57Bl6 mice (SMRTmRID) produces a novel respiratory distress syndrome (RDS) due to prematurity of the type I pneumocyte. Treatment with the anti-thyroid hormone drug, propylthiouracil (PTU), completely rescues the SMRT-induced RDS, suggesting an unrecognized and essential role for the thyroid hormone receptor (TR) in lung development. We show that TR and SMRT control type I pneumocyte differentiation through Klf2, which in turn appears to directly activate the type I pneumocyte gene program. Conversely, mice without lung Klf2 lack mature type I pneumocytes and die shortly after birth, closely recapitulating the SMRTmRID phenotype. These results identify a second nuclear receptor, the TR, in type I pneumocyte differentiation and suggest a new type of therapeutic option in the treatment of glucocorticoid non-responsive RDS.
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| Overall design |
Total RNA was obtained from WT and SMRT-RID E18.5 lungs of embryos from mothers treated with Diet containing 0.15% PTU or control chow for 2 days (from E16.5).
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| Contributor(s) |
Yu RT, Pei L, Evans RM |
| Citation(s) |
22001906 |
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| Submission date |
Jul 14, 2011 |
| Last update date |
Jun 14, 2018 |
| Contact name |
Ruth T Yu |
| Organization name |
Salk Institute
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| Department |
Gene Expression Lab
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| Lab |
Ronald Evans
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| Street address |
10010 N Torrey Pines Rd
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| City |
La Jolla |
| State/province |
CA |
| ZIP/Postal code |
92037 |
| Country |
USA |
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| Platforms (1) |
| GPL6885 |
Illumina MouseRef-8 v2.0 expression beadchip |
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| Samples (8)
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| GSM760579 |
normal wild-type E18.5 lungs, technical replicate 1 |
| GSM760580 |
normal wild-type E18.5 lungs, technical replicate 2 |
| GSM760581 |
SMRTmRID E18.5 lungs, technical replicate 1 |
| GSM760582 |
SMRTmRID E18.5 lungs, technical replicate 2 |
| GSM760583 |
normal wild-type E18.5 lungs with PTU, technical replicate 1 |
| GSM760584 |
normal wild-type E18.5 lungs with PTU, technical replicate 2 |
| GSM760585 |
SMRTmRID E18.5 lungs with PTU, technical replicate 1 |
| GSM760586 |
SMRTmRID E18.5 lungs with PTU, technical replicate 2 |
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| Relations |
| BioProject |
PRJNA144589 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE30661_RAW.tar |
3.1 Mb |
(http)(custom) |
TAR |
| GSE30661_non-normalized.txt.gz |
1.3 Mb |
(ftp)(http) |
TXT |
| Processed data included within Sample table |
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