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Loss of MBNL1 induces RNA misprocessing in the thymus and peripheral blood
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Loss of MBNL1 induces RNA misprocessing in the thymus and peripheral blood
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Journal Article
Loss of MBNL1 induces RNA misprocessing in the thymus and peripheral blood
2020
Overview
The thymus is a primary lymphoid organ that plays an essential role in T lymphocyte maturation and selection during development of one arm of the mammalian adaptive immune response. Although transcriptional mechanisms have been well documented in thymocyte development, co-/post-transcriptional modifications are also important but have received less attention. Here we demonstrate that the RNA alternative splicing factor MBNL1, which is sequestered in nuclear RNA foci by C(C)UG microsatellite expansions in myotonic dystrophy (DM), is essential for normal thymus development and function.
Mbnl1
129S1 knockout mice develop postnatal thymic hyperplasia with thymocyte accumulation. Transcriptome analysis indicates numerous gene expression and RNA mis-splicing events, including transcription factors from the TCF/LEF family.
CNBP
, the gene containing an intronic CCTG microsatellite expansion in DM type 2 (DM2), is coordinately expressed with
MBNL1
in the developing thymus and DM2 CCTG expansions induce similar transcriptome alterations in DM2 blood, which thus serve as disease-specific biomarkers.
The activity of the RNA splicing factor MBNL1 is altered in myotonic dystrophy (DM) patients. Here the authors characterize the thymic phenotype of
Mbnl1
knockout mice, including developmental defects, transcriptome changes, and RNA mis-splicing of transcripts encoding thymic transcription factors.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
/ 13/31
/ 38/77
/ 38/91
/ Adult
/ Aged
/ Animals
/ Blood
/ DNA-Binding Proteins - genetics
/ DNA-Binding Proteins - metabolism
/ Female
/ Humanities and Social Sciences
/ Humans
/ Male
/ Mice
/ Microsatellite Repeats - genetics
/ Myotonic Dystrophy - genetics
/ Myotonic Dystrophy - immunology
/ RNA
/ RNA-Binding Proteins - genetics
/ RNA-Binding Proteins - metabolism
/ RNA-Seq
/ Science
/ Splicing
/ Thymus
