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Cell surface and gene expression regulation molecules in dystrophinopathy: mdx vs. Duchenne Biol. Res.
FADIC,RICARDO.
Duchenne muscular dystrophy (DMD) is secondary to loss-of-function mutations in the dystrophin gene. The causes underlying the progression of DMD, differential muscle involvement, and the discrepancies in phenotypes among species with the same genetic defect are not understood. The mdx mouse, an animal model with dystrophin mutation, has a milder phenotype. This article reviews the available information on expression of signaling-related molecules in DMD and mdx. Extracellular matrix proteoglycans, growth factors, integrins, caveolin-3, and neuronal nitric oxide synthase expression do not show significant differences. Calcineurin is inconsistently activated in mdx, which is associated with lack of cardiomyopathy, compared to the permanent calcineurin...
Tipo: Journal article Palavras-chave: Dystrophinopathy; Signaling molecules; Duchenne muscular dystrophy.
Ano: 2005 URL: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602005000400010
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Effect of Citrus aurantium L. Essential Oil on Muscle Regeneration in Mdx Mice International Journal of Morphology
De Souza,Paula Aiello Tomé; Marques,Maria Júlia; Lima,Clélia Akiko Hiruma; Matheus,Selma Maria Michelin.
Duchenne muscular dystrophy (DMD) is a severe X-linked recessive disorder characterized by the progressive loss of muscular strength. Mdx mutant mice show a marked deficiency in dystrophin, which was related to muscle membrane stability. The aim of this study was to verify the possible protective anti-inflammatory effect of citrus oil on mdx muscle fibers. Thus, adult male and female mdx mice (014/06-CEEA) were divided into control and citrus-treated. After 60 days of treatment, one ml of blood was collected for creatine kinase (CK) test. Diaphragm, sternomastoideus, anterior tibial and gastrocnemius muscles were removed and processed according to histological routine methods. The observed alterations indicate a direct effect of citrus. Recent studies have...
Tipo: Journal article Palavras-chave: Muscle fiber; Duchenne muscular dystrophy; Citrus aurantium L; Mdx mouse; Muscle regeneration; Central nuclei; Creatine kinase.
Ano: 2011 URL: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0717-95022011000400048
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Histochemistry and Morphometric Analysis of Muscle Fibers from Patients with Duchenne Muscular Dystrophy (DMD) International Journal of Morphology
Cavalcanti,George Maciel; Oliveira,André de Sá Braga; Assis,Thiago de Oliveira; Chimelli,Leila Maria Cardão; Medeiros,Paloma Lys de; Mota,Diógenes Luís da.
The aim of the study was to analyze the muscle fibers by histochemistry and morphometric methods from patients with Duchenne muscular dystrophy (DMD). Muscle biopsies were taken from the vastus lateralis muscle of five boys between 13 and 15-years of age, with clinical diagnosis of DMD. The histochemistry was performed using myofibrillar ATPases (9.6, 4.6 and 4.3). To morphometrical analysis a computerized semiautomatic system and software Image-Lab was used. ATPase staining showed atrophy of muscle fibers. Fibrosis and adipose deposition occurred in variable degree depending of muscular involvement. The morphometrical analysis showed an increase of size (percentage) to type I fiber than other types in all patients. Furthermore, the type I fiber had a...
Tipo: Journal article Palavras-chave: Histochemistry analysis; Morphometric analysis; Vastus lateralis muscle; Duchenne muscular dystrophy.
Ano: 2011 URL: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0717-95022011000300046
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Impaired regeneration of dystrophin-deficient muscle fibers is caused by exhaustion of myogenic cells BJMBR
Luz,M.A.M.; Marques,M.J.; Santo Neto,H..
Duchenne muscular dystrophy is one of the most devastating myopathies. Muscle fibers undergo necrosis and lose their ability to regenerate, and this may be related to increased interstitial fibrosis or the exhaustion of satellite cells. In this study, we used mdx mice, an animal model of Duchenne muscular dystrophy, to assess whether muscle fibers lose their ability to regenerate after repeated cycles of degeneration-regeneration and to establish the role of interstitial fibrosis or exhaustion of satellite cells in this process. Repeated degenerative-regenerative cycles were induced by the injection of bupivacaine (33 mg/kg), a myotoxic agent. Bupivacaine was injected weekly into the right tibialis anterior muscle of male, 8-week-old mdx (N = 20) and...
Tipo: Info:eu-repo/semantics/other Palavras-chave: Mdx fiber regeneration; Bupivacaine; Duchenne muscular dystrophy; Muscle degeneration.
Ano: 2002 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2002000600009
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Patients with Duchenne and Becker muscular dystrophies are not more asymmetrical than healthy controls on timed performance of upper limb tasks BJMBR
Artilheiro,M.C.; Sá,C.S.C.; Fávero,F.M.; Caromano,F.A.; Voos,M.C..
This study aimed to investigate possible asymmetries and relationships between performance of dominant and non-dominant upper limbs (UL) in patients with Duchenne and Becker muscular dystrophies (DMD/BMD), to compare UL performance of patients and healthy subjects and to investigate the relationship between timed performance of UL and age, motor function and muscle strength in DMD/BMD patients. Sixteen patients with DMD and 3 with BMD were evaluated with Jebsen-Taylor Test (timed performance), Vignos scale and Dimension 3 of Motor Function Measure (motor function), and Medical Research Council scale (muscle strength) on a single session. ANOVA showed no asymmetry between dominant and non-dominant UL, except in the writing subtest, in patients and in...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Duchenne muscular dystrophy; Upper extremity; Evaluation; Physical therapy.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000800604
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Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans Ciência Rural
Moraes,Julieta Rodini Engrácia de; Malvestio,Lygia Maria Mouri; Martins,Isabela Mancini; Mosko,Patrícia Regina Erdmann; Engracia Filho,Jair Rodini; Moraes,Flávio Ruas de.
ABSTRACT: Golden Retriever muscular dystrophy (GRMD) is the most representative model for studying Duchenne muscular dystrophy (DMD) in humans, owing its phenotypic expression. DMD is a recessive disorder linked to the X chromosome in which the loss of dystrophin induces progressive weakness and degeneration of the skeletal and cardiac muscles, which lead to replacement by connective and adipose tissues. Onset of clinical signs occurs between 2 and 5 years of age, and many patients die from heart or respiratory failure. The main studies concerning dystrophic Golden Retrievers (DGR) sought to elucidate the pathophysiology of the disease and its clinical implications to develop therapies and alternative treatments to improve the quality of life and increase...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Duchenne muscular dystrophy; Animal model; Dystrophic Golden Retriever.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503
Registros recuperados: 6
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