Faber-Elmann A, Paas-Rozner M, Sela M, Mozes E
Department of Immunology, Weizmann Institute of Science, Rehovot 76100, Israel.
Proc Natl Acad Sci U S A. 1998 Nov 24;95(24):14320-5. doi: 10.1073/pnas.95.24.14320.
Myasthenia gravis (MG) is a T cell-regulated, antibody-mediated autoimmune disease. Two peptides representing sequences of the human acetylcholine receptor alpha-subunit, p195-212 and p259-271, previously were shown to stimulate the proliferation of peripheral blood lymphocytes of patients with MG and were found to be immunodominant T cell epitopes in SJL and BALB/c mice, respectively. Single amino acid-substituted analogs of p195-212 and p259-271, as well as a dual analog composed of the tandemly arranged two single analogs, were shown to inhibit, in vitro and in vivo, MG-associated autoimmune responses. Stimulation of T cells through the antigen-specific T cell receptor activates tyrosine kinases and phospholipase C (PLC). Therefore, in attempts to understand the mechanism of action of the analogs, we first examined whether the myasthenogenic peptides trigger tyrosine phosphorylation and activation of phospholipase C. For that purpose, we measured generation of inositol phosphates and tyrosine phosphorylation of PLC after stimulation of the p195-212- and p259-271-specific T cell lines with these myasthenogenic peptides. Both myasthenogenic peptides stimulated generation of inositol phosphates as well as tyrosine phosphorylation of PLC. However, the single and dual analogs, although inducing tyrosine phosphorylation of PLC, could not induce PLC activity. Furthermore, the single and dual analogs inhibited the induced PLC activity whereas they could not inhibit tyrosine phosphorylation of PLC that was caused by the myasthenogenic peptides. Thus, the altered peptides and the dual analog act as partial agonists. The down-regulation of PLC activity by the analogs may account for their capacity to inhibit in vitro MG-associated T cell responses.
重症肌无力(MG)是一种由T细胞调节、抗体介导的自身免疫性疾病。先前已证明,代表人类乙酰胆碱受体α亚基序列的两种肽,即p195 - 212和p259 - 271,可刺激MG患者外周血淋巴细胞的增殖,并且分别被发现是SJL和BALB/c小鼠中的免疫显性T细胞表位。p195 - 212和p259 - 271的单氨基酸取代类似物,以及由串联排列的两个单类似物组成的双类似物,已证明在体外和体内均可抑制MG相关的自身免疫反应。通过抗原特异性T细胞受体刺激T细胞会激活酪氨酸激酶和磷脂酶C(PLC)。因此,为了试图了解这些类似物的作用机制,我们首先检查了致重症肌无力肽是否会引发酪氨酸磷酸化和磷脂酶C的激活。为此,在用这些致重症肌无力肽刺激p195 - 212和p259 - 271特异性T细胞系后,我们测量了肌醇磷酸的生成以及PLC的酪氨酸磷酸化。两种致重症肌无力肽均刺激了肌醇磷酸的生成以及PLC的酪氨酸磷酸化。然而,单类似物和双类似物虽然可诱导PLC的酪氨酸磷酸化,但无法诱导PLC活性。此外,单类似物和双类似物抑制了诱导的PLC活性,而它们无法抑制由致重症肌无力肽引起的PLC的酪氨酸磷酸化。因此,改变后的肽和双类似物起部分激动剂的作用。类似物对PLC活性的下调可能解释了它们抑制体外MG相关T细胞反应的能力。