Results 31 to 40 of about 2,696 (156)

Dysfunctional lamins as mediators of oxidative stress in Emery-Dreifuss muscular dystrophy

open access: yesFolia Neuropathologica, 2017
Deficit of lamin A/C or emerin causes genetically transmitted Emery-Dreifuss muscular dystrophy (EDMD). As lamins are considered to be mediators of oxidative stress, the antioxidant/oxidant status was examined.
Irena Niebroj-Dobosz   +4 more
doaj   +1 more source

Novel LMNA Mutation in a Taiwanese Family with Autosomal Dominant Emery-Dreifuss Muscular Dystrophy

open access: yesJournal of the Formosan Medical Association, 2007
Emery-Dreifuss muscular dystrophy (EDMD) is characterized by early-onset contractures, slowly progressive weakness, and muscle wasting in humeroperoneal muscles, and adult-onset cardiomyopathy with conduction block. We analyzed blood samples from an EDMD
Wen-Chen Liang   +6 more
doaj   +1 more source

In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation

open access: yesCase Reports in Anesthesiology, 2012
Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy.
Frank Schuster   +7 more
doaj   +1 more source

Efficient Nonlinear Model Predictive Control of Automated Vehicles

open access: yesMathematics, 2022
In this paper, an efficient model predictive control (MPC) of velocity tracking of automated vehicles is proposed, in which a reference signal is given a priori.
Shuyou Yu   +5 more
doaj   +1 more source

Net39 protects muscle nuclei from mechanical stress during the pathogenesis of Emery-Dreifuss muscular dystrophy

open access: yesThe Journal of Clinical Investigation, 2023
Mutations in genes encoding nuclear envelope proteins lead to diseases known as nuclear envelopathies, characterized by skeletal muscle and heart abnormalities, such as Emery-Dreifuss muscular dystrophy (EDMD).
Yichi Zhang   +11 more
doaj   +1 more source

Development of accurate and tractable EDMD approximations for analysis and control [PDF]

open access: yes, 2023
peer reviewedThe Koopman operator is a theoretical framework that allows the use of linear analysis tools on transformations of nonlinear systems. Unfortunately, the analytical transformation of an arbitrary system is only possible for a specific family ...
Vande wouwer, Alain   +1 more
core   +1 more source

Cytoskeleton–Membrane Uncoupling in Duchenne Muscular Dystrophy: Implications for Newborn Screening and Early Protection

open access: yesCytoskeleton, EarlyView.
ABSTRACT The cytoskeleton of striated muscle integrates force transmission, mechanotransduction, and sarcolemmal stability through coordinated networks of sarcomeres, costameres, and intermediate filaments. Together, these systems establish mechanical continuity between the contractile apparatus, the sarcolemma, and the extracellular matrix.
Houda Cohen   +3 more
wiley   +1 more source

EDMD Collaboration and Virtual Prototyping [PDF]

open access: yes, 2014
Nokia Solutions and Networks (NSN) requires new tools for collaborating electronic and mechanic design phases in its product development. In the first part of this thesis will be introduced Electrical Data Mechanical Data (EDMD) application; the software
Välkky, Markus
core  

Loss of emerin alters myogenic signaling and miRNA expression in mouse myogenic progenitors. [PDF]

open access: yesPLoS ONE, 2012
Emerin is an integral membrane protein of the inner nuclear membrane. Mutations in emerin cause X-linked Emery-Dreifuss muscular dystrophy (EDMD), a disease characterized by skeletal muscle wasting and dilated cardiomyopathy.
Adam J Koch, James M Holaska
doaj   +1 more source

Microhomology-Mediated Nonhomologous End Joining Caused Rearrangement of EMD and FLNA in Emery-Dreifuss Muscular Dystrophy

open access: yesFrontiers in Genetics, 2021
Background: Emery–Dreifuss muscular dystrophy (EDMD) is a rare disease characterized by early joint contractures, slowly progressive muscular dystrophy, and cardiac involvement, which includes arrhythmia, dilated cardiomyopathy, hypertrophic ...
Danyu Song   +5 more
doaj   +1 more source

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