Solimena M, De Camilli P
Dept of Cell Biology, Yale University School of Medicine, New Haven, CT 06510.
Trends Neurosci. 1991 Oct;14(10):452-7. doi: 10.1016/0166-2236(91)90044-u.
Stiff-Man syndrome (SMS) is a disorder of the CNS, characterized by rigidity of the body musculature, which has been hypothesized to result from an impairment of GABAergic neurotransmission. GABA is the main inhibitory neurotransmitter of the brain. It is also a putative signal molecule in the pancreas, where it is produced by beta cells (insulin-secreting cells)--the autoimmune target in insulin-dependent diabetes mellitus (IDDM). Autoantibodies to the GABA-synthesizing enzyme glutamic acid decarboxylase (GAD) have been found in SMS and in IDDM. This review summarizes evidence suggesting that SMS may be an autoimmune disease and discusses the possible significance of the autoimmune response to GAD in SMS and IDDM.
僵人综合征(SMS)是一种中枢神经系统疾病,其特征为身体肌肉僵硬,据推测这是由γ-氨基丁酸(GABA)能神经传递受损所致。GABA是大脑主要的抑制性神经递质。它也是胰腺中的一种假定信号分子,由β细胞(分泌胰岛素的细胞)产生,而β细胞是胰岛素依赖型糖尿病(IDDM)的自身免疫靶点。在僵人综合征和胰岛素依赖型糖尿病中均发现了针对合成GABA的酶——谷氨酸脱羧酶(GAD)的自身抗体。本综述总结了表明僵人综合征可能是一种自身免疫性疾病的证据,并讨论了针对GAD的自身免疫反应在僵人综合征和胰岛素依赖型糖尿病中的可能意义。