Results 101 to 110 of about 59,671 (341)

Increased neointimal thickening in dystrophin-deficient mdx mice.

PLoS ONE, 2012
BackgroundThe dystrophin gene, which is mutated in Duchenne muscular dystrophy (DMD), encodes a large cytoskeletal protein present in muscle fibers. While dystrophin in skeletal muscle has been extensively studied, the function of dystrophin in vascular ...
Uwe Rauch, Annelie Shami, Feng Zhang, Virginie Carmignac, Madeleine Durbeej, Anna Hultgårdh-Nilsson   +5 more
doaj   +1 more source

CardiLect: A combined cross‐species lectin histochemistry protocol for the automated analysis of cardiac remodelling

ESC Heart Failure, Volume 12, Issue 2, Page 1398-1415, April 2025.
Abstract Background Cardiac remodelling, a crucial aspect of heart failure, is commonly investigated in preclinical models by quantifying cardiomyocyte cross‐sectional area (CSA) and microvascular density (MVD) via histological methods, such as immunohistochemistry.
Tamás G. Gergely, Tamás Kovács, Andrea Kovács, Viktória E. Tóth, Nabil V. Sayour, Gábor M. Mórotz, Csenger Kovácsházi, Gábor B. Brenner, Zsófia Onódi, Balázs Enyedi, Domokos Máthé, Przemyslaw Leszek, Zoltán Giricz, Péter Ferdinandy, Zoltán V. Varga   +14 more
wiley   +1 more source

How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse [PDF]

, 2015
Splice modulation therapy has shown great clinical promise in Duchenne muscular dystrophy, resulting in the production of dystrophin protein. Despite this, the relationship between restoring dystrophin to established dystrophic muscle and its ability to ...
Betts, C, Coursindel, T, El Andaloussi, S, Gait, M J, Godfrey, C, Hammond, S, Hildyard, J C W, McClorey, G, Muses, S, O'Donovan, L, Terry, R L, Wells, D J, Wells, K E, Wood, M J   +13 more
core   +2 more sources

What is the level of dystrophin expression required for effective therapy of Duchenne muscular dystrophy?

Journal of Muscle Research and Cell Motility, 2019
Duchenne muscular dystrophy (DMD) is a fatal X-linked muscle wasting disease. The disease is due to mutations in the DMD gene that encodes for a large intracellular protein called dystrophin.
D. Wells
semanticscholar   +1 more source

The Impact of Aquaporin‐4 Deletion on K+‐Induced Astrocytic Swelling Depends on K+ Concentration

Glia, EarlyView.
Swelling due to 10 mM [K+]o is reduced in astrocytes devoid of aquaporin‐4, while there is no difference between genotypes when exposed to 50 mM [K+]o. The two [K+]o concentrations induce distinct astrocytic Ca2+ signaling in the two genotypes pointing to a possible mechanistic basis of the differences observed. ABSTRACT Astrocytes swell in response to
Cecilie Bugge Bakketun, Lena Catherine Roth, Daniel Marelius Bjørnstad, Ole Petter Ottersen, Vidar Jensen, Rune Enger   +5 more
wiley   +1 more source

New insights into applications of base editor in hereditary disorders

Interdisciplinary Medicine, EarlyView.
Abstract Hereditary disorders are a group of diseases caused by genetic mutations or chromosomal variations. Although the incidence of each genetic disorder is relatively low, patients affected by the disease generally experience a range of severe symptoms, including blindness, disability, and even premature death. In addition, the available treatments
Maoping Cai, Linhui Zhang, Liqing Li, Yue Liu, Canbin Lv, Yan Shi, Jianyuan Zhao, Dingwei Ye, Yuanyuan Qu   +8 more
wiley   +1 more source

TNF-α-Induced microRNAs Control Dystrophin Expression in Becker Muscular Dystrophy

Cell Reports, 2015
The amount and distribution of dystrophin protein in myofibers and muscle is highly variable in Becker muscular dystrophy and in exon-skipping trials for Duchenne muscular dystrophy. Here, we investigate a molecular basis for this variability.
Alyson A. Fiorillo, Christopher R. Heier, James S. Novak, Christopher B. Tully, Kristy J. Brown, Kitipong Uaesoontrachoon, Maria C. Vila, Peter P. Ngheim, Luca Bello, Joe N. Kornegay, Corrado Angelini, Terence A. Partridge, Kanneboyina Nagaraju, Eric P. Hoffman   +13 more
doaj   +1 more source

Functional correction of dystrophin actin binding domain mutations by genome editing.

JCI Insight, 2017
Dystrophin maintains the integrity of striated muscles by linking the actin cytoskeleton with the cell membrane. Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene (DMD) that result in progressive, debilitating muscle ...
V. Kyrychenko, S. Kyrychenko, M. Tiburcy, J. Shelton, Chengzu Long, J. Schneider, W. Zimmermann, R. Bassel-Duby, E. Olson   +8 more
semanticscholar   +1 more source

Direct binding of Torpedo syntrophin to dystrophin and the 87 kDa dystrophin homologue [PDF]

FEBS Letters, 1995
Syntrophin, a 58‐kDa membrane‐associated protein, is one component of a protein complex associated with dystrophin and other members of the dystrophin family, including the 87‐kDa homologue (87K protein). To characterize interactions between syntrophin and 87K protein, we used an in vitro overlay binding assay.
Tim Dwyer, Tim Dwyer, Stanley C. Froehner   +2 more
openaire   +2 more sources

Overuse‐Induced Muscle Disorder: Establishing a Rat Model to Unravel the Role of Fibro‐Adipogenic Progenitor Cells in Intramuscular Fibrosis

Journal of Orthopaedic Research, EarlyView.
ABSTRACT Overuse‐induced muscle disorders (OIMD) frequently occur in athletes due to excessive and improper use under high physical demand, often leading to muscle pain and weakness. Limited studies have shown intramuscular fibrosis in OIMD, with fibro‐adipogenic progenitors (FAPs), also known as mesenchymal stromal cells (MSCs), playing a crucial role
Hiroyori Fusagawa, Tatsuya Sato, Azuma Naito, Nao Tokuda, Nao Yamauchi, Akiyoshi Uezumi, Madoka Uezumi, Yuki Saito, Minami Fusagawa, Hiroyuki Takashima, Nobutoshi Ichise, Toshifumi Ogawa, Takuro Karaushi, Noritsugu Tohse, Brian Feeley, Xuhui Liu, Atsushi Teramoto, Takashi Yamada   +17 more
wiley   +1 more source