重症肌无力中针对兰尼碱受体的自身免疫反应。

Autoimmunity against the ryanodine receptor in myasthenia gravis.

作者信息

Skeie G O, Lunde P K, Sejersted O M, Mygland A, Aarli J A, Gilhus N E

机构信息

Department of Neurology, University of Bergen, Bergen, Norway.

出版信息

Acta Physiol Scand. 2001 Mar;171(3):379-84. doi: 10.1046/j.1365-201x.2001.00841.x.

DOI:10.1046/j.1365-201x.2001.00841.x

PMID:11412151

Abstract

Some myasthenia gravis (MG) patients have antibodies against skeletal muscle antigens in addition to the acetylcholine receptor (AChR). A major antigen for these antibodies is the Ca2+ release channel of the sarcoplasmic reticulum the ryanodine receptor (RyR). These antibodies are found mainly in MG patients with a thymoma MG and correlate with severe MG symptoms. The antibodies recognize a region near the N-terminus on the RyR, which seems to be of importance for RyR regulation. The antibodies cause allosteric inhibition of RyR function in vitro, inhibiting Ca2+ release from sarcoplasmic reticulum.

摘要

一些重症肌无力（MG）患者除了产生抗乙酰胆碱受体（AChR）抗体外，还产生针对骨骼肌抗原的抗体。这些抗体的主要抗原是肌浆网的Ca2+释放通道——兰尼碱受体（RyR）。这些抗体主要在患有胸腺瘤的MG患者中发现，并且与严重的MG症状相关。这些抗体识别RyR上靠近N端的一个区域，该区域似乎对RyR的调节很重要。这些抗体在体外引起RyR功能的变构抑制，抑制肌浆网释放Ca2+。