Progressive Muscular Dystrophy in α-Sarcoglycan–deficient Mice

Progressive Muscular Dystrophy in α-Sarcoglycan–deficient Mice

Limb-girdle muscular dystrophy type 2D (LGMD 2D) is an autosomal recessive disorder caused by mutations in the alpha-sarcoglycan gene. To determine how alpha-sarcoglycan deficiency leads to muscle fiber degeneration, we generated and analyzed alpha-sarcoglycan- deficient mice. Sgca-null mice developed progressive muscular dystrophy and, in contrast to other animal models for muscular dystrophy,...

[Progressive muscular dystrophy].

This paper describes the case of a young man with progressive muscular dystrophy who had supraventricular arrhythmias and intractable congestive failure. Hemodynamic studies and left cineventriculographic findings were compatible with a diagnosis of severe left ventricular failure. Coronary cinearteriography showed an abrupt ending of the artery to the sino-atrial node. At necropsy, a noninflam...

Progressive Muscular Dystrophy (Erb.)

Genetic-epidemiological studies in progressive muscular dystrophy.

A genetic and epidemiological study is especially important in those hereditary diseases for which no effective treatment is known, since then genetic counselling is the only means of prevention. The progressive muscular dystrophies are typical conditions of this sort. Knowledge of the type of hereditary transmission for a given form of muscular dystrophy is the basis for correct genetic progno...

The electrocardiogram in Duchenne progressive muscular dystrophy.

Interpretation of the electrocardiogram in the Duchenne form of progressive muscular dystrophy (PMD) is still debated in the literature. The group studied consisted of 106 patients with the Duchenne type of PMD, aged 3 to 29 years. The duration of the disease varied from a few months to 15 years. One hundred one patients had abnormal electrocardiograms. These were of four types: type a (64 case...