Cytokines and Chemokines as Regulators of Skeletal Muscle Inflammation: Presenting the Case of Duchenne Muscular Dystrophy [PDF]
Mediators of Inflammation, 2013 Duchenne muscular dystrophy is a severe inherited muscle disease that affects 1 in 3500 boys worldwide. Infiltration of skeletal muscle by inflammatory cells is an important facet of disease pathophysiology and is strongly associated with disease ...
Boel De Paepe, Jan L. De Bleecker
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Therapeutic strategies to address neuronal nitric oxide synthase deficiency and the loss of nitric oxide bioavailability in Duchenne Muscular Dystrophy [PDF]
Orphanet Journal of Rare Diseases, 2017 Duchenne Muscular Dystrophy is a rare and fatal neuromuscular disease in which the absence of dystrophin from the muscle membrane induces a secondary loss of neuronal nitric oxide synthase and the muscles capacity for endogenous nitric oxide synthesis ...
Cara A. Timpani +2 more
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Cellular and animal models for facioscapulohumeral muscular dystrophy [PDF]
Disease Models & Mechanisms, 2020 Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common forms of muscular dystrophy and presents with weakness of the facial, scapular and humeral muscles, which frequently progresses to the lower limbs and truncal areas, causing profound
Alec M. DeSimone +3 more
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Tracking progress: an update on animal models for Duchenne muscular dystrophy [PDF]
Disease Models & Mechanisms, 2018 Duchenne muscular dystrophy (DMD) is a progressive, fatal, X-linked monogenic muscle disorder caused by mutations in the DMD gene. In order to test treatments for DMD, a range of natural and engineered animal models have been developed, including mice ...
Dominic J. Wells
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Clinical and genetic characterisation of dystrophin-deficient muscular dystrophy in a family of Miniature Poodle dogs [PDF]
, 2018 Four full-sibling intact male Miniature Poodles were evaluated at 4–19 months of age. One was clinically normal and three were affected. All affected dogs were reluctant to exercise and had generalised muscle atrophy, a stiff gait and a markedly elevated
A Aartsma-Rus +63 more
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iMyoblasts for ex vivo and in vivo investigations of human myogenesis and disease modeling
eLife, 2022 Skeletal muscle myoblasts (iMyoblasts) were generated from human induced pluripotent stem cells (iPSCs) using an efficient and reliable transgene-free induction and stem cell selection protocol.
Dongsheng Guo +15 more
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Animal Models of Muscular Dystrophy [PDF]
, 2012 The muscular dystrophies (MDs) represent a diverse collection of inherited human disorders, which affect to varying degrees skeletal, cardiac, and sometimes smooth muscle (Emery, 2002). To date, more than 50 different genes have been implicated as causing one or more types of MD (Bansal et al., 2003).
Rainer, Ng +10 more
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Muscular Dystrophy in Man and Animals [PDF]
Neurology, 1964 This volume of twelve chapters by multiple contributors is the most comprehensive treatment of the subject of muscular dystrophy and related disorders available to date. It is under the editorship of a worker in the field. Gross and microscopic pathology, findings on electron-microscopy, and the results of tissue culture studies are ...
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CRISPR-Generated Animal Models of Duchenne Muscular Dystrophy [PDF]
Genes, 2020 Duchenne muscular dystrophy (DMD) is a fatal X-linked recessive neuromuscular disorder most commonly caused by mutations disrupting the reading frame of the dystrophin (DMD) gene. DMD codes for dystrophin, which is critical for maintaining the integrity of muscle cell membranes.
Kenji Rowel Q. Lim +4 more
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Disease Models & Mechanisms, 2020 Dystrophin, encoded by the DMD gene on the X chromosome, stabilizes the sarcolemma by linking the actin cytoskeleton with the dystrophin-glycoprotein complex (DGC).
Naomi Teramoto +12 more
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