Results 51 to 60 of about 38,378 (273)

With Regard to the Expression Status of Sarcolemmal Aquaporin 4 in Human Muscular Dystrophies

Neurology and Clinical Neuroscience, EarlyView.
ABSTRACT Human muscular dystrophies are inherited muscle‐wasting diseases caused by the various kinds of gene mutations. Among them, Duchenne muscular dystrophy (DMD) is a representative type. Before the discovery of the causative dystrophin gene of DMD, the fragile myofiber plasma membrane was thought to be the trigger of myofiber necrosis in DMD ...
Yoshihiro Wakayama, Takahiro Jimi
wiley   +1 more source

Unraveling the spatial landscape of dystrophinopathies: a transcriptomic approach to Becker and Duchenne muscular dystrophies

The Journal of Pathology, EarlyView.
Abstract Dystrophinopathies are caused by pathogenic variants in the DMD gene, resulting in partial (Becker) or complete loss (Duchenne) of dystrophin. Becker (BMD) and Duchenne muscular dystrophy (DMD) are characterized by progressive muscle wasting, fatty replacement, fibrosis, and loss of function.
Laura GM Heezen, Qirong Mao, Stefan Nicolau, Claudio Novella Rausell, Julia van der Weerd, Jan Kueckelhaus, Rasya Gokul Nath, Jordi Diaz‐ Manera, Hermien E Kan, Erik H Niks, Maaike van Putten, Annemieke Aartsma‐Rus, Kevin M Flanigan, Ahmed Mahfouz, Pietro Spitali   +14 more
wiley   +1 more source

Impaired respiratory function in mdx and mdx/utrn^+/− mice [PDF]

Muscle & Nerve, 2011
AbstractMuscle fibrosis is a prominent pathological feature that directly causes muscle dysfunction in Duchenne muscular dystrophy (DMD). The DMD mouse models, mdx mice and mdx mice with haploinsufficiency of the utrophin gene (mdx/utrn+/−), display progressive diaphragm fibrosis.
Ping, Huang, Georgiana, Cheng, Haiyan, Lu, Mark, Aronica, Richard M, Ransohoff, Lan, Zhou   +5 more
openaire   +2 more sources

Importância do camundongo mdx na fisiopatologia da distrofia muscular de Duchenne The importance of mdx mouse in the pathophysiology of Duchenne's muscular distrophy

Arquivos de Neuro-Psiquiatria, 1997
O camundongo mdx desenvolve distrofia muscular recessiva ligada ao cromossoma X (locus Xp21.1) e não expressa distrofina. Embora não apresente intensa fibrose do tecido muscular e acúmulo de tecido adiposo, é considerado o modelo animal mais adequado da ...
Sandra Lopes Seixas, Jussara Lagrota-Cândido, Wilson Savino, Thereza Quirico-Santos   +3 more
doaj   +1 more source

Experimental investigations of synchrotron radiation at the onset of the quantum regime [PDF]

, 2012
The classical description of synchrotron radiation fails at large Lorentz factors, $\gamma$, for relativistic electrons crossing strong transverse magnetic fields $B$.
A. Dizdar, A. Mangiarotti, A. Sokolov, H. D. Thomsen, H. Knudsen, J. Esberg, J. S. Schwinger, K. K. Andersen, P. Sona, S. Ballestrero, T. J. Ketel, U. I. Uggerhøj, V. Baier, V. N. Baier, V. N. Baier   +14 more
core   +3 more sources

Proteomic Profiling of Myofiber Repair Annexins and Their Role in Duchenne Muscular Dystrophy

PROTEOMICS, EarlyView.
ABSTRACT Myofiber regeneration and membrane repair play crucial roles in maintaining the continuous physiological functioning of the neuromuscular system. A swift and efficient repair mechanism enables the rapid restoration of sarcolemmal integrity following cellular impairment in damaged skeletal muscles.
Paul Dowling, Dounia Bouragba, Elisa Negroni, Capucine Trollet, Margit Zweyer, Dieter Swandulla, Kay Ohlendieck   +6 more
wiley   +1 more source

Misplaced Golgi Elements Produce Randomly Oriented Microtubules and Aberrant Cortical Arrays of Microtubules in Dystrophic Skeletal Muscle Fibers

Frontiers in Cell and Developmental Biology, 2019
Differentiated mammalian cells and tissues, such as skeletal muscle fibers, acquire an organization of Golgi complex and microtubules profoundly different from that in proliferating cells and still poorly understood.
Sarah Oddoux, Davide Randazzo, Aster Kenea, Bruno Alonso, Kristien J. M. Zaal, Evelyn Ralston   +5 more
doaj   +1 more source

Altered muscle niche contributes to myogenic deficit in the D2-mdx model of severe DMD

Cell Death Discovery, 2023
Lack of dystrophin expression is the underlying genetic basis for Duchenne muscular dystrophy (DMD). However, disease severity varies between patients, based on specific genetic modifiers. D2-mdx is a model for severe DMD that exhibits exacerbated muscle
Davi A. G. Mázala, Ravi Hindupur, Young Jae Moon, Fatima Shaikh, Iteoluwakishi H. Gamu, Dhruv Alladi, Georgiana Panci, Michèle Weiss-Gayet, Bénédicte Chazaud, Terence A. Partridge, James S. Novak, Jyoti K. Jaiswal   +11 more
doaj   +1 more source

Targeting a therapeutic LIF transgene to muscle via the immune system ameliorates muscular dystrophy. [PDF]

, 2019
Many potentially therapeutic molecules have been identified for treating Duchenne muscular dystrophy. However, targeting those molecules only to sites of active pathology is an obstacle to their clinical use.
Bertoni, Carmen, Flores, Ivan, Ramos, Julian, Tidball, James G, Wang, Ying, Wehling-Henricks, Michelle, Welc, Steven S   +6 more
core  

MIMO radar with broadband waveforms: Smearing filter banks and 2D virtual arrays [PDF]

, 2009
In this paper MIMO radars with broadband waveforms are considered. A time domain viewpoint is taken, which allows frequency invariant beamforming with a filter bank called the smearing filter bank.
Chen, Chun-Yang, Pal, Piya, Vaidyanathan, P. P.   +2 more
core   +2 more sources