Blechynden L M, Lawson M A, Tabarias H, Garlepp M J, Sherman J, Raben N, Lawson C M
Australian Neuromuscular Research Institute and Department of Medicine, University of Western Australia, Queen Elizabeth II Medical Centre, Nedlands.
Hum Gene Ther. 1997 Aug 10;8(12):1469-80. doi: 10.1089/hum.1997.8.12-1469.
Polymyositis is regarded as an autoimmune inflammatory muscle disease. A major subgroup of patients have autoantibodies to cellular histidyl-transfer RNA synthetase (HRS). We have analyzed the role of the autoantigen HRS in the induction of murine myositis in a comparative study of inoculation of BALB/c mice with recombinant HRS protein versus naked DNA coding for HRS. Adult BALB/c mice produced antibodies to human HRS following inoculation with HRS protein and adjuvant, but myositis was not observed. Alternatively, expression plasmid DNA constructs encoding full-length and truncated human HRS were inoculated intramuscularly in gene transfer studies. DNA-inoculated mice produced relatively low anti-HRS antibody titers. However, in contrast to recombinant HRS protein-inoculated mice, HRS gene transfer induced pathology with evidence of cellular infiltration of perivascular and endomysial regions of the inoculated muscle. Multiple inoculations of a plasmid construct encoding a hybrid molecule consisting of HRS and the transferrin receptor cytoplasmic tail induced the highest levels of antibodies and persisting cellular infiltration. Unlike HRS, expression of influenza virus hemagglutinin (HA) following inoculation of an HA plasmid did not induce myositis. Transfer of naked DNA constructs expressing HRS is likely to provide valuable information on the autoimmune response to this protein and its role in the development of myositis.
多发性肌炎被视为一种自身免疫性炎性肌肉疾病。一大类患者具有针对细胞组氨酰 - 转运RNA合成酶(HRS)的自身抗体。我们在一项比较研究中分析了自身抗原HRS在诱导小鼠肌炎中的作用,该研究将重组HRS蛋白与编码HRS的裸DNA接种到BALB/c小鼠体内。成年BALB/c小鼠接种HRS蛋白和佐剂后产生了针对人HRS的抗体,但未观察到肌炎。另外,在基因转移研究中,将编码全长和截短型人HRS的表达质粒DNA构建体进行肌肉内接种。接种DNA的小鼠产生的抗HRS抗体滴度相对较低。然而,与接种重组HRS蛋白的小鼠不同,HRS基因转移诱导了病理学变化,接种肌肉的血管周围和肌内膜区域有细胞浸润的证据。多次接种编码由HRS和转铁蛋白受体细胞质尾组成的杂交分子的质粒构建体诱导了最高水平的抗体和持续的细胞浸润。与HRS不同,接种HA质粒后流感病毒血凝素(HA)的表达未诱导肌炎。表达HRS的裸DNA构建体的转移可能会为针对该蛋白的自身免疫反应及其在肌炎发展中的作用提供有价值的信息。