基础医学各论I-骨骼肌(英文).pptVIP

How can gastrocnemius contraction result in two different movements? The lever system of muscles and bones: Here, muscle contraction must generate 70 kg force to hold a 10 kg object that is 30 cm away from the site of muscle attachment. Muscle contraction that moves the attachment site on bone 1 cm results in a 7 cm movement of the object 30 cm away from the site; similar gains in movement velocity occur. Duchenne muscular dystrophy（Duchenne型肌营养不良） weakens the hip and trunk muscles, thus altering the lever-system relationships of the muscles and bones that are used to stand up. Duchenne muscular dystrophy is a devastating, progressive disease that occurs in boys; it is characterized by the progressive necrosis of skeletal muscle fibers and death at an average age of 16, usually from respiratory failure. It is the second most common genetic disorder in humans, and there is no specific treatment. The course of the disease includes slow muscular development, progressive weakness, and frequent contractures. The disease is usually recognized at ages 2 to 5, and the child is usually confined to a wheelchair by age 12. Laboratory observations show highly elevated serum concentrations of creatine kinase and other soluble sarcoplasmic enzymes. Both fast and slow muscle fibers are affected by fiber necrosis and phagocytosis, balanced by marked regeneration of cells in the early stages of the disease. The fibers resemble fetal muscles, in terms of their isoenzyme patterns, with marked dedifferentiation. Fiber death and replacement by fat and connective tissue gradually predominate. DNA analysis reveals that the disease is caused by the deficiency of a gene on the X chromosome. The product of the gene is a cytoskeletal protein called dystrophin that forms a network adjacent to the sarcolemma. Dystrophin is a very large protein (426 kDa). It is a minor constituent of muscle and links sarcomeres to the sarcolemma via association with a glycoprotein inserted into the membrane.