PASSIVE TRANSFER OF SERONEGATIVE MYASTHENIA-GRAVIS TO MICE

被引:63
作者
BURGES, J
VINCENT, A
MOLENAAR, PC
NEWSOMDAVIS, J
PEERS, C
WRAY, D
机构
[1] UNIV LEEDS, DEPT PHARMACOL, LEEDS LS2 9JT, W YORKSHIRE, ENGLAND
[2] ROYAL FREE HOSP, SCH MED, NEUROSCI GRP, LONDON, ENGLAND
[3] JOHN RADCLIFFE HOSP, INST MOLEC MED, OXFORD OX3 9DU, ENGLAND
[4] LEIDEN UNIV, DEPT PHYSIOL, DIV MEMBRANE PHYSIOL & PHARMACOL, LEIDEN, NETHERLANDS
关键词
MYASTHENIA GRAVIS; ANTIBODIES; ACETYLCHOLINE; NEUROMUSCULAR JUNCTION;
D O I
10.1002/mus.880171208
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Muscle weakness in myasthenia gravis is due to autoantibody-induced loss of functional acetylcholine receptors (AChR). About 15% of myasthenia gravis patients, however, do not have detectable anti-AChR antibodies. To investigate the effect of their plasma immunoglobulins on neuromuscular transmission, mice were injected with plasma (and in some cases purified immunoglobulin G (IgG)) from 7 ''seronegative'' myasthenia gravis (SMG) patients, and neuromuscular transmission parameters were examined. When injected for 15 days, all patients' plasma caused reductions in miniature endplate potential amplitudes, while endplate potential quantal content was significantly reduced by plasma from 4 of the 7 patients. There were no changes in ACh-induced depolarization or single channel properties, and I-125-alpha-bungarotoxin binding studies showed no effect on AChR number, except in 1 case. Purified IgG injected for 3 days had similar effects to plasma injected for 15 days. Our findings confirm that SMG is autoantibody mediated and that there are pathogenic IgG antibodies. SMG appears to be a heterogeneous disorder and the target(s) for the antibodies may be diverse. (C) 1994 John Wiley & Sons, Inc.
引用
收藏
页码:1393 / 1400
页数:8
相关论文
共 40 条
[1]  
BARRETTJOLLE R, IN PRESS PFLUGERS AR
[2]   A MYASTHENIA-GRAVIS PLASMA IMMUNOGLOBULIN REDUCES MINIATURE ENDPLATE POTENTIALS AT HUMAN ENDPLATES INVITRO [J].
BURGES, J ;
WRAY, DW ;
PIZZIGHELLA, S ;
HALL, Z ;
VINCENT, A .
MUSCLE & NERVE, 1990, 13 (05) :407-413
[3]  
BURGES J, 1987, J PHYSIOL-LONDON, V394, pP148
[4]   RELEASE OF TRANSMITTER AT NORMAL, MYASTHENIA-GRAVIS AND MYASTHENIC SYNDROME AFFECTED HUMAN ENDPLATES [J].
CULLCANDY, SG ;
MILEDI, R ;
TRAUTMANN, A ;
UCHITEL, OD .
JOURNAL OF PHYSIOLOGY-LONDON, 1980, 299 (FEB) :621-638
[5]   QUANTAL COMPONENTS OF THE END-PLATE POTENTIAL [J].
DELCASTILLO, J ;
KATZ, B .
JOURNAL OF PHYSIOLOGY-LONDON, 1954, 124 (03) :560-573
[6]  
DOLLY JO, 1988, J PHYSIOL-LONDON, V399, P577
[7]   MYASTHENIA-GRAVIS - IMMUNOBIOLOGY OF A RECEPTOR DISORDER [J].
DRACHMAN, DB .
TRENDS IN NEUROSCIENCES, 1983, 6 (11) :446-451
[8]  
DRACHMAN DB, 1987, ANN NY ACAD SCI, V505, P90
[9]   ELECTROPHYSIOLOGICAL INVESTIGATION OF NEURO-MUSCULAR TRANSMISSION IN MYASTHENIA GRAVIS [J].
ELMQVIST, D ;
KUGELBERG, J ;
HOFMANN, WW ;
QUASTEL, DMJ .
JOURNAL OF PHYSIOLOGY-LONDON, 1964, 174 (03) :417-&
[10]   ULTRASTRUCTURAL LOCALIZATION OF ACETYLCHOLINE-RECEPTOR IN MYASTHENIA-GRAVIS AND IN ITS EXPERIMENTAL AUTOIMMUNE MODEL [J].
ENGEL, AG ;
LINDSTROM, JM ;
LAMBERT, EH ;
LENNON, VA .
NEUROLOGY, 1977, 27 (04) :307-315