myotonic dystrophy/carbohydrate

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Carbohydrate metabolism and insulin resistance in myotonia dystrophica.

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Carbohydrate metabolism was studied in fourteen patients with myotonia dystrophica (MD) using oral glucose, fructose and galactose tolerance tests. Insulin responses to tolbutamide, glucagon, arginine and leucine were determined and insulin resistance was measured with exogenous iv insulin. Glucose

Carbohydrate composition of erythrocyte membranes and glycosidase activities in serum in patients with myotonic dystrophy, limb-girdle dystrophy and congenital myotonia.

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A number of abnormalities in cell membrane function, including cells other than muscle cells, have been described in patients with inherited muscular diseases such as myotonic dystrophy and congenital myotonia. The basic molecular defects are, however, still unknown. The complex carbohydrates of

Carbohydrate metabolism in dystrophia myotonica.

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Serum insulin, blood sugar, and growth hormone levels were measured in response to a 50g oral glucose tolerance test in 10 patients with proven dystrophia myotonica. Three patients belonged to one family; seven patients had no known family history of the disease. One patient, a chronic invalid aged

Carbohydrate metabolism in patients with dystrophia myotonica.

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Carbohydrate and lipid metabolism in relation to body composition in myotonic dystrophy.

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Metabolic implications of distal atrophy. Carbohydrate metabolism in centronuclear myopathy.

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Centronuclear myopathy, like myotonic dystrophy, is characterized by muscle wasting and type 1 fiber atrophy. To determine whether this disorder might include a derangement in carbohydrate metabolism similar to that in myotonic dystrophy, 3 comparably wasted patients with centronuclear myopathy,

Sialic acid content in serum IgG from patients with myotonic dystrophy compared with healthy controls.

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One of the determinants of the lifetime of circulating glycoproteins is their content of the terminal carbohydrate sialic acid. In order to elucidate a possible mechanism behind reduced concentration of IgG in patients with myotonic dystrophy, serum IgG was isolated by affinity chromatography and

[Multiorgan manifestations in myotonic dystrophy type 1].

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Myotonic dystrophy is one of the most common, autosomal dominantly inherited adult-onset muscle disorders. Two types of the disease are known: type 1 is characterized by distal weakness and myotonia, but type 2 is associated with proximal weakness and milder clinical course. It is also called as

Metabolic studies in muscular dystrophy: a role for insulin.

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Insulin is important in maintaining carbohydrate tolerance and normal muscle mass. Cabohydrate intolerance and muscle wasting are frequent in the neuromuscular disorders associated with endocrinopathy and in myotonic dystrophy. Studies of the systemic factors that regulate insulin release in

Skeletal muscle disorders and associated factors that limit exercise performance.

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The study of skeletal muscle disorders is providing potentially important insights into regulatory mechanisms in human exercise and fatigue and information useful for diagnostic and treatment purposes. This review primarily concerned the general metabolic and physiological factors which set upper

Precise Targeted Cleavage of a r(CUG) Repeat Expansion in Cells by Using a Small-Molecule-Deglycobleomycin Conjugate.

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RNA repeat expansions cause more than 30 neurological and neuromuscular diseases with no known cures. Since repeat expansions operate via diverse pathomechanisms, one potential therapeutic strategy is to rid them from disease-affected cells, using bifunctional small molecules that cleave the

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