MBNL expression in autoregulatory feedback loops

A flow cytometry-based screen identifies MBNL1 modulators that rescue splicing defects in myotonic dystrophy type I

(2017) Fan Zhang et al.   HUMAN MOLECULAR GENETICS

Distal Alternative Last Exons Localize mRNAs to Neural Projections

(2016) J. Matthew Taliaferro et al.   MOLECULAR CELL

Autoregulation of MBNL1 function by exon 1 exclusion from MBNL1 transcript

(2016) Patryk Konieczny et al.   NUCLEIC ACIDS RESEARCH

Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila

(2016) Estefania Cerro-Herreros et al.   Scientific Reports

Staufen1 Regulates Multiple Alternative Splicing Events either Positively or Negatively in DM1 Indicating Its Role as a Disease Modifier

(2016) Emma Bondy-Chorney et al.   PLoS Genetics

Phenylbutazone induces expression of MBNL1 and suppresses formation of MBNL1-CUG RNA foci in a mouse model of myotonic dystrophy

(2016) Guiying Chen et al.   Scientific Reports

Myotonic dystrophies: An update on clinical aspects, genetic, pathology, and molecular pathomechanisms

(2015) Giovanni Meola et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins

(2015) Eric T. Wang et al.   GENOME RESEARCH

Progress and prospects of gene therapy clinical trials for the muscular dystrophies

(2015) Niclas E. Bengtsson et al.   HUMAN MOLECULAR GENETICS

Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disorders

(2014) John Douglas Cleary et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

ABLIM1splicing is abnormal in skeletal muscle of patients with DM1 and regulated by MBNL, CELF and PTBP1

(2014) Natsumi Ohsawa et al.   GENES TO CELLS

Nuclear localization of MBNL1: splicing-mediated autoregulation and repression of repeat-derived aberrant proteins

(2014) Yoshihiro Kino et al.   HUMAN MOLECULAR GENETICS

circRNA Biogenesis Competes with Pre-mRNA Splicing

(2014) Reut Ashwal-Fluss et al.   MOLECULAR CELL

Myotonic Dystrophy

(2014) Charles A. Thornton   NEUROLOGIC CLINICS

MBNL proteins and their target RNAs, interaction and splicing regulation

(2014) Patryk Konieczny et al.   NUCLEIC ACIDS RESEARCH

RBFOX1 Cooperates with MBNL1 to Control Splicing in Muscle, Including Events Altered in Myotonic Dystrophy Type 1

(2014) Roscoe Klinck et al.   PLoS One

Compound loss of muscleblind-like function in myotonic dystrophy

(2013) Kuang-Yung Lee et al.   EMBO Molecular Medicine

Progressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice

(2013) Michael G. Poulos et al.   HUMAN MOLECULAR GENETICS

MBNL1 and PTB cooperate to repress splicing of Tpm1 exon 3

(2013) Clare Gooding et al.   NUCLEIC ACIDS RESEARCH

Transcriptome-wide Regulation of Pre-mRNA Splicing and mRNA Localization by Muscleblind Proteins

(2012) Eric T. Wang et al.   CELL

Neuronal regulation of pre-mRNA splicing by polypyrimidine tract binding proteins, PTBP1 and PTBP2

(2012) Niroshika Keppetipola et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

The splicing regulator Rbfox2 is required for both cerebellar development and mature motor function

(2012) L. T. Gehman et al.   GENES & DEVELOPMENT

The myotonic dystrophies: molecular, clinical, and therapeutic challenges

(2012) Bjarne Udd et al.   LANCET NEUROLOGY

RNA Interference Targeting CUG Repeats in a Mouse Model of Myotonic Dystrophy

(2012) Krzysztof Sobczak et al.   MOLECULAR THERAPY

Muscleblind-like 2-Mediated Alternative Splicing in the Developing Brain and Dysregulation in Myotonic Dystrophy

(2012) Konstantinos Charizanis et al.   NEURON

Muscleblind-Like 1 Knockout Mice Reveal Novel Splicing Defects in the Myotonic Dystrophy Brain

(2012) Koichi Suenaga et al.   PLoS One

CUGBP1 and MBNL1 preferentially bind to 3′ UTRs and facilitate mRNA decay

(2012) Akio Masuda et al.   Scientific Reports

Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of CaV1.1 calcium channel

(2011) Zhen Zhi Tang et al.   HUMAN MOLECULAR GENETICS

Analysis of Exonic Regions Involved in Nuclear Localization, Splicing Activity, and Dimerization of Muscleblind-like-1 Isoforms

(2011) Hélène Tran et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Autoregulated Splicing ofmuscleblind-like 1 (MBNL1) Pre-mRNA

(2011) Devika P. Gates et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The splicing regulator Rbfox1 (A2BP1) controls neuronal excitation in the mammalian brain

(2011) Lauren T Gehman et al.   NATURE GENETICS

Misregulated alternative splicing of BIN1 is associated with T tubule alterations and muscle weakness in myotonic dystrophy

(2011) Charlotte Fugier et al.   NATURE MEDICINE

A conserved motif controls nuclear localization of Drosophila Muscleblind

(2010) Juan M. Fernandez-Costa et al.   MOLECULES AND CELLS

Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy

(2010) Hongqing Du et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Conserved developmental alternative splicing of muscleblind-like (MBNL) transcripts regulates MBNL localization and activity

(2010) Fulvia Terenzi et al.   RNA Biology

Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy

(2009) Robert J. Osborne et al.   HUMAN MOLECULAR GENETICS

Auto- and Cross-Regulation of the hnRNP L Proteins by Alternative Splicing

(2009) O. Rossbach et al.   MOLECULAR AND CELLULAR BIOLOGY

MBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1

(2009) Yoshihiro Kino et al.   NUCLEIC ACIDS RESEARCH

Autoregulation of Fox protein expression to produce dominant negative splicing factors

(2009) A. Damianov et al.   RNA

Structural insights into RNA recognition by the alternative-splicing regulator muscleblind-like MBNL1

(2008) Marianna Teplova et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart

(2008) A. Kalsotra et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA