Dystrophin Dp71 and the neuropathophysiology of Duchenne muscular dystrophy

M Naidoo, K Anthony - Molecular Neurobiology, 2020 - Springer
Duchenne muscular dystrophy (DMD) is caused by frameshift mutations in the DMD gene
that prevent the body-wide translation of its protein product, dystrophin. Besides a severe …

The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction

DGS Wilson, A Tinker, T Iskratsch - Communications Biology, 2022 - nature.com
Dystrophin is the central protein of the dystrophin-glycoprotein complex (DGC) in skeletal
and heart muscle cells. Dystrophin connects the actin cytoskeleton to the extracellular matrix …

Dystrophin Dp71: the smallest but multifunctional product of the Duchenne muscular dystrophy gene

R Tadayoni, A Rendon, LE Soria-Jasso… - Molecular …, 2012 - Springer
Dystrophin Dp71 is expressed in all tissues, with the exception of skeletal muscle, and is the
main Duchenne muscular dystrophy (DMD) gene product in brain. As full-length dystrophin …

Dystrophins, utrophins, and associated scaffolding complexes: role in mammalian brain and implications for therapeutic strategies

C Perronnet, C Vaillend - BioMed Research International, 2010 - Wiley Online Library
Two decades of molecular, cellular, and functional studies considerably increased our
understanding of dystrophins function and unveiled the complex etiology of the cognitive …

Linear motifs: lost in (pre) translation

RJ Weatheritt, TJ Gibson - Trends in biochemical sciences, 2012 - cell.com
Pretranslational modification by alternative splicing, alternative promoter usage and RNA
editing enables the production of multiple protein isoforms from a single gene. A large …

Nonmechanical roles of dystrophin and associated proteins in exercise, neuromuscular junctions, and brains

B Nichols, S Takeda, T Yokota - Brain sciences, 2015 - mdpi.com
Dystrophin-glycoprotein complex (DGC) is an important structural unit in skeletal muscle that
connects the cytoskeleton (f-actin) of a muscle fiber to the extracellular matrix (ECM) …

[HTML][HTML] A role for β-dystroglycan in the organization and structure of the nucleus in myoblasts

IA Martínez-Vieyra, A Vásquez-Limeta… - … et Biophysica Acta (BBA …, 2013 - Elsevier
We recently characterized a nuclear import pathway for β-dystroglycan; however, its nuclear
role remains unknown. In this study, we demonstrate for the first time, the interaction of β …

Dp71 expression in human glioblastoma

S Ruggieri, M De Giorgis, T Annese, R Tamma… - International journal of …, 2019 - mdpi.com
Background: Dp71 is the most abundant dystrophin (DMD) gene product in the nervous
system. Mutation in the Dp71 coding region is associated with cognitive disturbances in …

Knockdown of dystrophin Dp71 impairs PC12 cells cycle: localization in the spindle and cytokinesis structures implies a role for Dp71 in cell division

M Villarreal-Silva, F Centeno-Cruz… - PLoS …, 2011 - journals.plos.org
The function of dystrophin Dp71 in neuronal cells remains to be established. Previously, we
revealed the involvement of this protein in both nerve growth factor (NGF)-induced neuronal …

Ablation of the dystrophin Dp71f alternative C-terminal variant increases sarcoma tumour cell aggressiveness

N Alnassar, J Hajto, RMH Rumney… - Human Molecular …, 2024 - academic.oup.com
Alterations in Dp71 expression, the most ubiquitous dystrophin isoform, have been
associated with patient survival across tumours. Intriguingly, in certain malignancies, Dp71 …