The hallmarks of myotonic dystrophy type 1 muscle dysfunction

LL Ozimski, M Sabater‐Arcis, A Bargiela… - Biological …, 2021 - Wiley Online Library
ABSTRACT Myotonic dystrophy type 1 (DM1) is the most prevalent form of muscular
dystrophy in adults and yet there are currently no treatment options. Although this disease …

Alternative splicing as a biomarker and potential target for drug discovery

K Le, BS Prabhakar, W Hong, L Li - Acta Pharmacologica Sinica, 2015 - nature.com
Alternative splicing is a key process of multi-exonic gene expression during pre-mRNA
maturation. In this process, particular exons of a gene will be included within or excluded …

Myotonic dystrophy type 1: role of CCG, CTC and CGG interruptions within DMPK alleles in the pathogenesis and molecular diagnosis

M Santoro, M Masciullo, G Silvestri, G Novelli… - Clinical …, 2017 - Wiley Online Library
Myotonic dystrophy type 1 (DM1) is a multisystem neuromuscular disease caused by a CTG
triplet expansion in the 3′‐untranslated region (3′‐UTR) of DMPK gene. This CTG array is …

iPSC-derived cardiomyocytes from patients with myotonic dystrophy type 1 have abnormal ion channel functions and slower conduction velocities

H Poulin, A Mercier, M Djemai, V Pouliot… - Scientific reports, 2021 - nature.com
Cardiac complications such as electrical abnormalities including conduction delays and
arrhythmias are the main cause of death in individuals with Myotonic Dystrophy type 1 …

Mitochondrial apoptosis is induced by Alkoxy phenyl-1-propanone derivatives through PP2A-mediated dephosphorylation of Bad and Foxo3A in CLL

MA Pagano, E Tibaldi, P Molino, F Frezzato, V Trimarco… - Leukemia, 2019 - nature.com
Abstract Protein phosphatase 2 A (PP2A) is a tumour suppressor whose strong inhibition
underlies the phosphorylation-dependent, anti-apoptotic mechanisms in Chronic …

MBNL splicing activity depends on RNA binding site structural context

K Taylor, ŁJ Sznajder, P Cywoniuk… - Nucleic acids …, 2018 - academic.oup.com
Muscleblind-like (MBNL) proteins are conserved RNA-binding factors involved in alternative
splicing (AS) regulation during development. While AS is controlled by distribution of MBNL …

Protein phosphorylation alterations in myotonic dystrophy type 1: a systematic review

A Costa, AC Cruz, F Martins, S Rebelo - International journal of molecular …, 2023 - mdpi.com
Among the most common muscular dystrophies in adults is Myotonic Dystrophy type 1
(DM1), an autosomal dominant disorder characterized by myotonia, muscle wasting and …

Small molecules which improve pathogenesis of myotonic dystrophy type 1

M López-Morató, JD Brook… - Frontiers in neurology, 2018 - frontiersin.org
Myotonic dystrophy type 1 (DM1) is the most common muscular dystrophy in adults for which
there is currently no treatment. The pathogenesis of this autosomal dominant disorder is …

Block or degrade? Balancing on-and off-target effects of antisense strategies against transcripts with expanded triplet repeats in DM1

N El Boujnouni, ML van der Bent, M Willemse… - … Therapy-Nucleic Acids, 2023 - cell.com
Antisense oligonucleotide (ASO) therapies for myotonic dystrophy type 1 (DM1) are based
on elimination of transcripts containing an expanded repeat or inhibition of sequestration of …

Pluripotent stem cells in disease modeling and drug discovery for myotonic dystrophy type 1

N Bérenger-Currias, C Martinat, S Baghdoyan - Cells, 2023 - mdpi.com
Myotonic dystrophy type 1 (DM1) is a progressive multisystemic disease caused by the
expansion of a CTG repeat tract within the 3′ untranslated region (3′ UTR) of the …