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Duchenne Muscular Dystrophy

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Cover of 'Duchenne Muscular Dystrophy'

Table of Contents

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    Book Overview
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    Chapter 1 An Overview of Recent Therapeutics Advances for Duchenne Muscular Dystrophy
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    Chapter 2 Clinical Manifestations and Overall Management Strategies for Duchenne Muscular Dystrophy
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    Chapter 3 Cardiac Involvement in Duchenne Muscular Dystrophy and Related Dystrophinopathies
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    Chapter 4 Characterization of the Inflammatory Response in Dystrophic Muscle Using Flow Cytometry
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    Chapter 5 Imaging Analysis of the Neuromuscular Junction in Dystrophic Muscle
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    Chapter 6 System Biology Approach: Gene Network Analysis for Muscular Dystrophy
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    Chapter 7 Proteomic Profiling of the Dystrophin-Deficient Brain
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    Chapter 8 Probing the Pathogenesis of Duchenne Muscular Dystrophy Using Mouse Models
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    Chapter 9 Exon Skipping Therapy Using Phosphorodiamidate Morpholino Oligomers in the mdx52 Mouse Model of Duchenne Muscular Dystrophy
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    Chapter 10 Designing Effective Antisense Oligonucleotides for Exon Skipping
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    Chapter 11 Identification of Splicing Factors Involved in DMD Exon Skipping Events Using an In Vitro RNA Binding Assay
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    Chapter 12 The Use of Antisense Oligonucleotides for the Treatment of Duchenne Muscular Dystrophy
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    Chapter 13 PMO Delivery System Using Bubble Liposomes and Ultrasound Exposure for Duchenne Muscular Dystrophy Treatment
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    Chapter 14 Proton Nuclear Magnetic Resonance (1H NMR) Spectroscopy-Based Analysis of Lipid Components in Serum/Plasma of Patients with Duchenne Muscular Dystrophy (DMD)
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    Chapter 15 Test of Antifibrotic Drugs in a Cellular Model of Fibrosis Based on Muscle-Derived Fibroblasts from Duchenne Muscular Dystrophy Patients
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    Chapter 16 Flow Cytometry-Defined CD49d Expression in Circulating T-Lymphocytes Is a Biomarker for Disease Progression in Duchenne Muscular Dystrophy
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    Chapter 17 Advanced Methods to Study the Cross Talk Between Fibro-Adipogenic Progenitors and Muscle Stem Cells
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    Chapter 18 AAV6 Vector Production and Purification for Muscle Gene Therapy
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    Chapter 19 From gRNA Identification to the Restoration of Dystrophin Expression: A Dystrophin Gene Correction Strategy for Duchenne Muscular Dystrophy Mutations Using the CRISPR-Induced Deletion Method
  21. Altmetric Badge
    Chapter 20 Erratum to: Functional Analysis of Membrane Proteins Produced by Cell-Free Translation
Attention for Chapter 3: Cardiac Involvement in Duchenne Muscular Dystrophy and Related Dystrophinopathies
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Chapter title
Cardiac Involvement in Duchenne Muscular Dystrophy and Related Dystrophinopathies
Chapter number 3
Book title
Duchenne Muscular Dystrophy
Published in
Methods in molecular biology, January 2018
DOI 10.1007/978-1-4939-7374-3_3
Pubmed ID
Book ISBNs
978-1-4939-7373-6, 978-1-4939-7374-3
Authors

Sophie I. Mavrogeni, George Markousis-Mavrogenis, Antigoni Papavasiliou, George Papadopoulos, Genovefa Kolovou

Abstract

Dystrophinopathies include Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy (XLCM), and facioscapulohumeral muscular dystrophy (FSHD). DMD/BMD are X-linked recessive disorders, related to the synthesis of dystrophin. Most of DMD after the third decade of their age develop cardiomyopathy that remains silent, due to relative physical inactivity. Cardiac disease in female carriers presents with hypertrophy, arrhythmias or dilated cardiomyopathy, clinically overt by increasing age.In ECG, DMD presents increased R/S ratio in the right precordial leads, deep Q waves in the lateral leads, conduction abnormalities, and arrhythmias. Echocardiography, although widely available and inexpensive, is highly depended on the acoustic window and operator's experience. Tissue Doppler can be used to identify early changes of cardiomyopathy and detect progressive cardiac damage. CMR, a noninvasive, nonradiating technique, by evaluation of cardiac volumes, mass, ejection fraction, inflammation, and fibrosis, is ideal for early diagnosis. Subepicardial fibrosis in the inferolateral wall is the typical CMR lesion in DMD/BMD.Early initiation of angiotensin converting enzyme inhibitors (ACEI) treatment, such as perindopril, was associated with lower mortality in DMD with normal LV ejection fraction at study entry. Other studies documented that a beta-blocker (BB), in addition to ACEI, improves LV systolic function in MD. These encouraging results recommend initiation of ACEI and/or BB early after diagnosis of the muscular dystrophy, especially in DMD.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 56 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 56 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 7 13%
Other 7 13%
Student > Postgraduate 5 9%
Student > Doctoral Student 4 7%
Student > Ph. D. Student 4 7%
Other 8 14%
Unknown 21 38%
Readers by discipline Count As %
Medicine and Dentistry 18 32%
Biochemistry, Genetics and Molecular Biology 7 13%
Neuroscience 3 5%
Agricultural and Biological Sciences 2 4%
Sports and Recreations 1 2%
Other 3 5%
Unknown 22 39%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 28 October 2017.
All research outputs
#18,574,814
of 23,006,268 outputs
Outputs from Methods in molecular biology
#7,961
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Outputs of similar age
#330,463
of 442,258 outputs
Outputs of similar age from Methods in molecular biology
#950
of 1,498 outputs
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