Muscular Diseases

Gene delivery constitutes an interesting approach to treat diseased striated muscles. Administration of pDNA coding for the dystrophin protein has been extensively studied in animal models of Duchenne or Becker muscular dystrophy and even evaluated in Phase 1 clinical trials. Although most of the cationic vehicles appeared to be relatively inefficient in promoting transgenic expression in the muscle tissue, D-SPM polycations were reported to show a transient increase in reporter gene expression after direct intramuscular injection in skeletal muscles with maximum efficiency. 

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L. K. was supported by a grant from the Swiss Society for Research on Muscular Diseases awarded to U.A. and Sergei Strelkov. This work was also supported by grants from the NCCR Nanoscale Science, the Swiss National Science Foundation, and by the M. E. Muller Foundation. 

Diseases of muscle are usually classified as muscular dystrophies, inflammatory or congenital myopathies, metabolic disorders affecting the muscle, and neurological diseases affecting the innervation of the muscle (so-called motor neuron diseases). In the past, the energy metabolism of some muscular diseases... 

Pain, muscular weakness, cramps and ease of fatigue are the most usual symptoms of muscular disease. In most cases, it is diseases of the vascular or nervous system or problems with the processes providing energy within the muscle that are responsible for clinical problems with muscles. Other clinical problems include the muscular dystrophies, myotonic disorders, inflammatory myopathies and disorders of neuromuscular transmission (see Walton, 1996). The best known is Duchenne muscular dystrophy. 

Heart stroke, endotoxins or haemorrhagic Serious anaemia Intensive muscular disease... 

Brody disease (BD, OMIM 601003) was first decribed by Dr. Irwin A. Brody in 1969 (Brody, 1969). It is a rare genetic muscular disease which can be transmitted in an autosomal recessive or dominant manner. The disease is genetically heterogeneous, and ATP2A1 mutations have been identified in recessive forms of the disease only (Odermatt et al., 1996). 

M16. Morrell, R. M., Abnormal hepatic tests in muscular disease. Preliminary report. A.M.A. Arch. Intern. Med. 104, 99 (1959). 

Extrahepatic causes of increased transaminases include (1.) cardiac infarction, (2.) severe tachycardia, (3.) muscular diseases, (4.) pulmonary embolism, (5.) hypo-/ hyperthyreosis, 6.) coeliac disease, (7.) heatstroke, (8.) hyperthermia, (9.) excessive physical exercise, (10.) longterm fasting, (11.) above-average protein intake, (12.) haemolysis, (13.) postprandial and (14.) long-term venous stasis. 

Serious infectious diseases including HIV-related diseases and tuberculosis Serious neurological and muscular diseases Serious renal diseases Serious respiratory diseases Serious skin disorders Sexually transmitted diseases... 

Becker PE 1971 Genetic approaches to the nosology of muscular disease myotonias and similar diseases. Birth Defects Orig Artie Ser 7 52—62... 

The interrelations between vitamin E and selenium in cattle and sheep are undoubtedly as complex as they are in other species. It seems reasonable to state that vitamin E, in combatting the toxicity of unsaturated fat, acts as an antioxidant, for its effect can be duplicated by many other antioxidants and redox dyestuffs. Similarly it is indisputable that selenium is a dietar essential for ruminants and that its absence from their diet results in muscular disease. Both unsaturated fat excess and selenium deficiency must produce primary disturbances in the muscle cells. These disturbances need not be common to both, for muscle reacts similarly to a variety of biochemical insult. In the presence of selenium and the absence of unsaturated fat, vitamin E requirements of ruminants appear to be extremely small. The failure to produce reproductive disorders in ruminants by experimental vitamin E deficiency, and the failure to produce muscular disease on fat-free diets deficient in vitamin E but likely to have been adequate in selenium content is evidence of this contention. How vitamin E acts in preventing muscular disease due to selenium deficiency, however, is not known, and this aspect needs elucidation. 

Hasegawa, Y., Oura, S., Takahashi, J. Exoskeletal meal assistance system (EMAS II) for patients with progressive muscular disease. Advanced Robotics 27(18), 1385-1398... 

In muscular disease, needle EMG waveforms vary in strength, waveshape, and frequency content. For example, in muscular dystrophy, the EMG action events are low in amplitude, short in duration (1 to 2 ms), and high in frequency (up to 40 per second). When the bioelectric signals are introduced into a loudspeaker so that they can be heard, there is a high-pitched characteristic sound. These diseased muscles fatigue easily, and this is reflected in their action events that are greatly reduced with sustained contraction. 

The effects of non-muscular diseases and injury on muscle metabolism have been investigated. Oxidative muscle metabolism has been assessed in people with spinal cord injuries. The rate of PCr resynthesis in right vastus lateralis muscle was measured after electrical stimulation for 60 s at... 

According to the National Research Council, National Academy of Sciences, "One could reasonably suppose that selenium is involved in such human medical problems as cancer, cataracts, diseases of the liver, cardiovascular or muscular diseases, and the aging process." Additional health problems which appear to result from a selenium deficiency are discussed in this section, also. 

Examination of CSP is important in many nenro-muscular disease patients, such as ones in whom an aspect of peripheral neuropathy, a motor neuron disorder, or a CNS component is suspected. Wrong... 

Disruption of intracellular structure and the proper transmission and signaling of forces can contribute to multiple muscular diseases. In the case of Duchenne muscular dystrophy, progressive muscle... 

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