Inclusion body myositis, muscle blood vessel and cardiac amyloidosis, and transthyretin Val122Ile allele
Inclusion body myositis, muscle blood vessel and cardiac amyloidosis, and transthyretin Val122Ile allele
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DOI：
10.1002/1531-8249(200004)47:4
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发表时间：
2000-04-01
影响因子：
11.2
通讯作者：
Vidal, R
中科院分区：
文献类型：
--
作者：
Askanas, V;Engel, WK;Vidal, R
典型的散发性包涵体肌炎肌肉活检是空泡化的肌纤维，含有细胞内淀粉样沉积物和“阿尔茨海默特征性”蛋白质的积累。没有肌肉血管或心脏淀粉样变性。我们报告了一个70岁的非洲裔美国人，其甲状腺素运载蛋白Val 122 Ile等位基因为纯合子，同时患有散发性包涵体肌炎和心脏淀粉样变性。他独特的病态。特征包括在突出的肌肉血管淀粉样蛋白和空泡化肌纤维内的嗜中性淀粉样蛋白沉积物中的甲状腺素运载蛋白免疫反应性。
Typical of sporadic inclusion body myositis muscle biopsies are vacuolated muscle fibers containing intracellular amyloid deposits and accumulations of "Alzheimer-characteristic" proteins. There is no muscle blood vessel or cardiac amyloidosis. We report on a 70-year-old African-American mao homozygous for the transthyretin Val122Ile allele who has both sporadic inclusion body myositis and cardiac amyloidosis. His unique pathological. features included transthyretin immunoreactivity in prominent muscle blood vessel amyloid and congophilic amyloid deposits within vacuolated muscle fibers.