Results 91 to 100 of about 7,799 (249)

Functional characterisation of filamentous actin probe expression in neuronal cells. [PDF]

PLoS ONE, 2017
Genetically encoded filamentous actin probes, Lifeact, Utrophin and F-tractin, are used as tools to label the actin cytoskeleton. Recent evidence in several different cell types indicates that these probes can cause changes in filamentous actin dynamics,
Shrujna Patel, Sandra Y Y Fok, Holly Stefen, Tamara Tomanić, Esmeralda Parić, Rosanna Herold, Merryn Brettle, Aleksandra Djordjevic, Thomas Fath   +8 more
doaj   +1 more source

Obligatory and accessory respiratory muscle structure, function and control in early and advanced disease in the mdx mouse model of Duchenne muscular dystrophy

The Journal of Physiology, Volume 603, Issue 14, Page 4113-4152, 15 July 2025.
Abstract figure legend We examined obligatory (diaphragm, external intercostal and parasternal) and accessory (sternomastoid, cleidomastoid, scalene and trapezius) muscle form, function and control in early (4 months) and advanced (16 months) dystrophic disease. Peak inspiratory pressure is preserved in 4‐month‐old mdx mice but is decreased in 16‐month‐
Aoife D. Slyne, David P. Burns, Karina Wöller, Amandine May, Roisin Dowd, Sarah E. Drummond, Grzegorz Jasionek, Ken D. O'Halloran   +7 more
wiley   +1 more source

Skeletal muscle-specific ablation of gamma(cyto)-actin does not exacerbate the mdx phenotype.

PLoS ONE, 2008
We previously documented a ten-fold increase in gamma(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased gamma(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response.
Kurt W Prins, Dawn A Lowe, James M Ervasti   +2 more
doaj   +1 more source

Dystrophin and utrophin: the missing links!

FEBS Letters, 1995
There is considerable sequence homology between dystrophin and utrophin, both at the protein and DNA level, and consequently it was assumed that their domain structures and functions would be similar. As more of the detailed biochemical and cell biological properties of these two proteins become known, so it becomes clear that there are subtle if not ...
Winder, S.J., Gibson, T.J., Kendrick-Jones, J.   +2 more
openaire   +2 more sources

Subtle Neuromuscular Defects in Utrophin-deficient Mice [PDF]

The Journal of Cell Biology, 1997
Utrophin is a large cytoskeletal protein that is homologous to dystrophin, the protein mutated in Duchenne and Becker muscular dystrophy. In skeletal muscle, dystrophin is broadly distributed along the sarcolemma whereas utrophin is concentrated at the neuromuscular junction.
R M, Grady, J P, Merlie, J R, Sanes
openaire   +2 more sources

UDP‐glucose dehydrogenase variants cause dystroglycanopathy

Annals of Clinical and Translational Neurology, Volume 12, Issue 6, Page 1302-1308, June 2025.
Abstract UDP‐glucose dehydrogenase (UGDH) variants have been associated with hypotonia, developmental delay, and epilepsy. We report the first pathologic evidence of dystroglycanopathy in siblings with UGDH variants. Both presented around 6 months with developmental delay and elevated creatinine kinase.
Anna M. Reelfs, Carrie M. Stephan, Theresa M. Czech, Mary O. Cox, Soumya Joseph, Benjamin W. Darbro, Steven A. Moore, Kevin P. Campbell, Katherine D. Mathews   +8 more
wiley   +1 more source

Utrophin up-regulation by an artificial transcription factor in transgenic mice.

PLoS ONE, 2007
Duchenne Muscular Dystrophy (DMD) is a severe muscle degenerative disease, due to absence of dystrophin. There is currently no effective treatment for DMD.
Elisabetta Mattei, Nicoletta Corbi, Maria Grazia Di Certo, Georgios Strimpakos, Cinzia Severini, Annalisa Onori, Agata Desantis, Valentina Libri, Serena Buontempo, Aristide Floridi, Maurizio Fanciulli, Dilair Baban, Kay E Davies, Claudio Passananti   +13 more
doaj   +1 more source

Post-natal induction of PGC-1α protects against severe muscle dystrophy independently of utrophin [PDF]

, 2014
Background: Duchenne muscle dystrophy (DMD) afflicts 1 million boys in the US and has few effective treatments. Constitutive transgenic expression of the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator (PGC)-1α ...
Arany, Zolt, Chan, Mun Chun, Farrell, Caitlin, Patten, Ian S, Raghuram, Srilatha, Rowe, Glenn C   +5 more
core   +1 more source

The Heterotaxy Gene CCDC11 Is Important for Cytokinesis via RhoA Regulation

Cytoskeleton, Volume 82, Issue 6, Page 360-371, June 2025.
ABSTRACT Mutations in CCDC11 (cfap53) have been identified in multiple patients with heterotaxy (Htx), a disorder of left–right (LR) patterning of the internal organs. In Xenopus, depletion of Ccdc11 causes defects in LR patterning, recapitulating the patient phenotype. Upon Ccdc11 depletion, monociliated cells of the Left–Right Organizer (LRO) exhibit
Saurabh S. Kulkarni, Rachel E. Stephenson, Sarah Amalraj, Angelo Arrigo, Ewelina Betleja, James J. Moresco, John R. Yates III, Moe R. Mahjoub, Ann L. Miller, Mustafa K. Khokha   +9 more
wiley   +1 more source

Efficacy of Prednisolone in Generated Myotubes Derived From Fibroblasts of Duchenne Muscular Dystrophy Patients

Frontiers in Pharmacology, 2018
Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy characterized by progressive muscle degeneration. This disease is caused by the mutation or deletion of the dystrophin gene. Currently, there are no effective treatments
Tsubasa Kameyama, Tsubasa Kameyama, Kazuki Ohuchi, Kazuki Ohuchi, Michinori Funato, Shiori Ando, Shiori Ando, Satoshi Inagaki, Satoshi Inagaki, Arisu Sato, Arisu Sato, Junko Seki, Chizuru Kawase, Kazuhiro Tsuruma, Ichizo Nishino, Shinsuke Nakamura, Masamitsu Shimazawa, Takashi Saito, Shin’ichi Takeda, Hideo Kaneko, Hideaki Hara   +20 more
doaj   +1 more source