Richards R B, Passmore I K, Dempsey E F
Animal Health Laboratory, Department of Agriculture, South Perth, Western Australia.
Acta Neuropathol. 1988;77(1):95-99. doi: 10.1007/BF00688248.
Fiber-type proportions were determined in 12 skeletal muscles (peronaeus tertius, tibialis cranialis, tensor fascia lata, psoas major, extensor digitorum lateralis, tensor fascia antibrachii, vastus intermedius, soleus, anconaeus, and flexor digitorum superficialis of fore and hind limb) from infantile (20 weeks), juvenile (16 months) and adult (2.5 and 3.5 years) Merino sheep with ovine congenital progressive muscular dystrophy and in age-matched controls. Although confined to type I fibers, lesions were severe not only in type I fiber dominant muscles (vastus intermedius, soleus and anconaeus) but also in the type II dominant medial triceps brachii and in the superficial digital flexors which had approximately equal proportions of both fiber types. The frequency distribution curves of myofiber diameter in dystrophic anconaeus and superficial digital flexor of the fore limb altered as the disease progressed. In young sheep (20 weeks) type I fibers increased in size, then in juvenile and adult sheep the curves became flatter and broader and lost symmetry due to the presence of hypertrophic and atrophic fibers. The fiber diameter profiles were similar to those seen in Duchenne dystrophy in humans.
在患有绵羊先天性进行性肌营养不良的婴儿期（20周）、幼年期（16个月）和成年期（2.5岁和3.5岁）美利奴绵羊以及年龄匹配的对照羊的12块骨骼肌（第三腓骨肌、胫骨前肌、阔筋膜张肌、腰大肌、外侧趾长伸肌、前臂筋膜张肌、股中间肌、比目鱼肌、肘肌以及前后肢的指浅屈肌）中测定了纤维类型比例。虽然病变仅限于I型纤维，但不仅在以I型纤维为主的肌肉（股中间肌、比目鱼肌和肘肌）中严重，而且在以II型纤维为主的肱三头肌内侧头以及两种纤维类型比例大致相等的指浅屈肌中也很严重。随着疾病进展，患营养不良症的肘肌和前肢指浅屈肌的肌纤维直径频率分布曲线发生了变化。在幼羊（20周）中，I型纤维尺寸增大，然后在幼年和成年羊中，由于存在肥大和萎缩纤维，曲线变得更平坦、更宽阔且失去对称性。纤维直径分布与人类杜兴氏肌营养不良症中的相似。
