myotonic dystrophy/carbohydrate

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

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

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

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,

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

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

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

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

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