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首页> 外文学位 >CLINICAL, GENETIC AND MORPHOLOGICAL CHARACTERIZATION OF CONGENITAL MYASTHENIA GRAVIS IN THE SMOOTH FOX TERRIER (PATHOLOGY).
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CLINICAL, GENETIC AND MORPHOLOGICAL CHARACTERIZATION OF CONGENITAL MYASTHENIA GRAVIS IN THE SMOOTH FOX TERRIER (PATHOLOGY).

机译：先天性重症肌无力狗的临床，遗传和形态学特征（病理）。

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摘要

In 13 smooth fox terriers with a congenital form of myasthenia gravis, clinical signs included intermittent, progressive muscle weakness that became more pronounced with exercise; muscle wasting; megaesophagus; and aspiration pneumonia. Neurologic abnormalities were apparent only during periods of weakness and included inability to retract the forelimbs and hindlimbs from painful stimuli. A decrement of the compound muscle action potential was evident during repetitive supramaximal nerve stimulation. Intravenous injection of a short-acting cholinesterase inhibitor evoked immediate improvement of clinical and electromyographic signs. Antibodies directed against acetylcholine receptors were not demonstrable in serum and were not bound to acetylcholine receptors in muscle. The amount of acetylcholine receptor protein in biopsied external intercostal muscles from 9 affected pups was less than 25% of the amount in 5 unaffected littermates. The later finding accounted for the reduction in amplitude of miniature end-plate potential and the failure of neuromuscular transmission. Treatment with a long-acting cholinesterase inhibitor in 6 cases resulted in temporary improvement in muscle strength.;Ultrastructural changes present within the neuromuscular junction included a decreased number of synaptic vesicles, decreased postsynaptic membrane length, decreased postsynaptic membrane area, and a decrease in the ratio of postsynaptic membrane length to the presynaptic membrane length. There was a significant decrease in the mean fiber diameter of Type I muscle fibers of the biceps femoris and type I and II muscle fibers of the biceps brachii in the affected dogs as compared to their littermate controls.;The phenotypes with respect to congenital myasthenia gravis of 132 smooth fox terrier dogs from 25 matings were analyzed by both prospective and retrospective matings. It was determined that congenital myasthenia gravis in the smooth fox terrier dog breed is inherited in an autosomal recessive manner with complete penetrance. The symbol mg for the gene for congenital myasthenia gravis in the smooth fox terrier is proposed. Atempts to maintain live affected dogs to adulthood were unsuccessful and it is concluded that this is a lethal trait.
机译：在13例先天性重症肌无力平滑狐犬中，临床体征包括间歇性进行性肌肉无力，随着运动而变得更加明显。肌肉消瘦;食道和吸入性肺炎。神经系统异常仅在无力时才出现，包括无法从疼痛刺激中收回前肢和后肢。重复的超最大神经刺激过程中，复合肌肉动作电位明显降低。静脉内注射短效胆碱酯酶抑制剂可立即改善临床和肌电图征象。针对乙酰胆碱受体的抗体在血清中未显示，并且未与肌肉中的乙酰胆碱受体结合。来自9只患病幼仔的活检的肋间外肌中乙酰胆碱受体蛋白的含量少于5个未患同窝仔的数量的25％。后来的发现解释了微型终板电位振幅降低和神经肌肉传递失败的原因。用长效胆碱酯酶抑制剂治疗6例，肌肉力量暂时改善。;神经肌肉接头内的超微结构改变包括突触小泡数量减少，突触后膜长度减少，突触后膜面积减少以及神经突触膜面积减少。突触后膜长度与突触前膜长度之比。与同龄对照相比，患犬的股二头肌的I型肌纤维和肱二头肌的I型和II型肌纤维的平均纤维直径显着降低；与先天性重症肌无力有关的表型通过前瞻性和回顾性交配分析了来自25个交配的132只光滑狐狸梗犬。已确定，光滑狐狸狗品种中的先天性重症肌无力是通过常染色体隐性遗传并完全外显而遗传的。提出了在光滑的狐狸梗中先天性重症肌无力基因的符号mg。将活着的受感染的狗维持成年的尝试是不成功的，并且得出结论，这是一种致命的特征。

著录项

  • 作者

    MILLER, LISA MAAG.;

  • 作者单位

    Washington State University.;

  • 授予单位 Washington State University.;
  • 学科 Biology Veterinary Science.
  • 学位 Ph.D.
  • 年度 1984
  • 页码 165 p.
  • 总页数 165
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类
  • 关键词

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