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muscular dystrophies/albumin

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Dystrophin analysis in Duchenne and Becker muscular dystrophy carriers: correlation with intracellular calcium and albumin.

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Immunocytochemical localization and immunoblot analysis of dystrophin in muscle fibers of 11 obligate and probable, and 7 possible carriers of Duchenne and Becker muscular dystrophy revealed an abnormal expression of the protein in 3 of them. Localization of calcium and albumin, as endogenous
Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201

Nonmuscular involvement in merosin-negative congenital muscular dystrophy.

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The spectrum of nonmuscular involvement in six children with merosin-negative congenital muscular dystrophy is described. In all children, biochemical, neuroradiologic, cardiac, and neurophysiologic studies were performed. Cerebral structures that were myelinated at gestation, including internal

Albumin targeting of damaged muscle fibres in the mdx mouse can be monitored by MRI.

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Increased sarcolemmal permeability has been implicated as a major pathological event in muscular dystrophies. In our study, we evaluated whether damaged muscle fibres can be specifically targeted using albumin as a carrier. We tagged human serum albumin (HSA) with Gadolinium (Gd) and systemically

Decreased resting energy expenditure in patients with Duchenne muscular dystrophy.

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BACKGROUND Skeletal muscle metabolism is a major determinant of resting energy expenditure (REE). Although the severe muscle loss that characterizes Duchenne muscular dystrophy (DMD) may alter REE, this has not been extensively investigated. METHODS We studied REE in 77 patients with DMD ranging in
We have characterized the time course of muscle pathology development during the postnatal maturation of quadriceps and tibialis anterior muscle in dystrophic golden retriever dogs. We determined the percentages of degenerating, regenerating, calcium-positive, hypercontracted, albumin-positive, and

Decrease of lactoferrin concentration in the tears of myotonic muscular dystrophy patients.

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Tears from myotonic muscular dystrophy (MMD) patients and normal controls were analyzed for their tear proteins by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Lactoferrin comprised about 18% of the total tear protein in MMD patients as opposed to about 27% in normals. The albumin
Serial consecutive frozen sections from 164 muscle biopsy specimens were studied cytochemically. We localized calcium and albumin as endogenous markers of extracellular fluid penetration and C3 and C9 complement components as markers of muscle fiber necrosis. In both Duchenne dystrophy and

Contrast agent-enhanced magnetic resonance imaging of skeletal muscle damage in animal models of muscular dystrophy.

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Membrane lesions play an early role in the pathogenesis of muscular dystrophy. Using a new albumin-targeted contrast agent (MS-325), sarcolemmal integrity of two animal models for muscular dystrophy was studied by MRI. Intravenously injected MS-325 does not enter skeletal muscle of normal mice.

Treatment with human immunoglobulin G improves the early disease course in a mouse model of Duchenne muscular dystrophy.

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Duchenne muscular dystrophy (DMD) is a severe hereditary myopathy. Standard treatment by glucocorticosteroids is limited because of numerous side effects. The aim of this study was to test immunomodulation by human immunoglobulin G (IgG) as treatment in the experimental mouse model (mdx) of DMD. 2

Elevated levels of albumin in soleus and diaphragm muscles of mdx mice.

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Muscle damage is often associated with an influx of extracellular fluid containing albumin into the muscle. Muscles affected by muscular dystrophy undergo severe muscle damage; therefore, the hypothesis was tested that muscles of dystrophic (mdx) mice contain elevated levels of albumin. Albumin

[Hemorrheological survey in dystrophia myotonica and other muscular dystrophies].

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We have determined the main hemorheological parameters in a group of patients suffering from myotonic dystrophy and in another group with oculopharyngeal or limb-girdle myopathy. From the results we have obtained it is evident that of all the rheological parameters considered only hematocrit,

[Proteinemia in young cattle during nutrition-induced muscular dystrophy].

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The origin and course of enzootic white muscle disease affecting young cattle in spring months after transfer from stables to pastures were accompanied by an apparent tendency to dysproteinemia. Clinically diseased animals showed a decrease of total protein in the blood serum and considerable
In order to evaluate the natural course of Duchenne's cardiomyopathy (DMD), 201T1-SPECT and RI cardioangiography with 99mTc-albumin were performed in 14 patients. They were examined once a year for five years except for 6 patients. Hypo-perfusion were observed in both posterior-inferior and anterior

Stimulation of calcineurin signaling attenuates the dystrophic pathology in mdx mice.

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Utrophin has been studied extensively in recent years in an effort to find a cure for Duchenne muscular dystrophy. In this context, we previously showed that mice expressing enhanced muscle calcineurin activity (CnA*) displayed elevated levels of utrophin around their sarcolemma. In the present
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