TY - JOUR
T1 - Pathophysiology of myasthenia gravis with antibodies to the acetylcholine receptor, muscle-specific kinase and low-density lipoprotein receptor-related protein 4
AU - Verschuuren, Jan J. G. M.
AU - Huijbers, Maartje G.
AU - Plomp, Jaap J.
AU - Niks, Erik H.
AU - Molenaar, Peter C.
AU - Martinez-Martinez, Pilar
AU - Gomez, Alejandro M.
AU - De Baets, Marc H.
AU - Losen, Mario
PY - 2013/7
Y1 - 2013/7
N2 - Myasthenia gravis is caused by antibodies to the acetylcholine receptor, muscle-specific kinase, low-density lipoprotein receptor-related protein 4, or possibly yet unidentified antibodies. The mechanisms by which these antibodies interfere with the function of postsynaptic proteins include complement activation, antigenic modulation by crosslinking of the target proteins, competition with ligand binding sites, or steric hindrance which inhibits conformational changes or binding to associated proteins. Screening for auto-antibodies to different postsynaptic targets, and also for low-affinity antibodies, is contributing to a more accurate diagnosis of MG patients. Further studies into the specific pathophysiological pathways of the several MG subforms might help to develop new, more antigen specific, therapies.
AB - Myasthenia gravis is caused by antibodies to the acetylcholine receptor, muscle-specific kinase, low-density lipoprotein receptor-related protein 4, or possibly yet unidentified antibodies. The mechanisms by which these antibodies interfere with the function of postsynaptic proteins include complement activation, antigenic modulation by crosslinking of the target proteins, competition with ligand binding sites, or steric hindrance which inhibits conformational changes or binding to associated proteins. Screening for auto-antibodies to different postsynaptic targets, and also for low-affinity antibodies, is contributing to a more accurate diagnosis of MG patients. Further studies into the specific pathophysiological pathways of the several MG subforms might help to develop new, more antigen specific, therapies.
KW - Myasthenia gravis
KW - Neuromuscular junction
KW - MuSK
KW - AChR
KW - Lrp4
U2 - 10.1016/j.autrev.2013.03.001
DO - 10.1016/j.autrev.2013.03.001
M3 - Article
C2 - 23535160
SN - 1568-9972
VL - 12
SP - 918
EP - 923
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
IS - 9
ER -