중증 근무력증에서 일어나는 신경근접합부의 면역병리학적 변화
Immunopathologic Change of Neuromuscular Junction in Myasthenia Gravis
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초록
The most essential phenomenon of myasthenia gravis (MG) is the impaired neuromuscular transmission caused by autoantibodies directed to proteins in the postsynaptic muscle membrane of the neuromuscular junction (NMJ). It is a highly specialized structure, which is designed to facilitate a chemical transmission from a motor nerve to a skeletal muscle. The integrity and efficiency of NMJ is attributed to a systematic interaction between acetylcholine receptor (AChR) and other synaptic proteins. Immunolopathologic process induced by autoantibodies attacks other AChR-associated synaptic proteins as well as the AChRs. Although the loss of the AChRs is a key feature of MG, the damage of AChR-associated synaptic proteins aggravates the pathologic process and delays the repair process. Therefore, ideally, the goal of therapy should be to improve the potential restoration process of NMJ as well as discontinue the abnormal autoimmune process.
Abstract
The most essential phenomenon of myasthenia gravis (MG) is the impaired neuromuscular transmission caused by autoantibodies directed to proteins in the postsynaptic muscle membrane of the neuromuscular junction (NMJ). It is a highly specialized structure, which is designed to facilitate a chemical transmission from a motor nerve to a skeletal muscle. The integrity and efficiency of NMJ is attributed to a systematic interaction between acetylcholine receptor (AChR) and other synaptic proteins. Immunolopathologic process induced by autoantibodies attacks other AChR-associated synaptic proteins as well as the AChRs. Although the loss of the AChRs is a key feature of MG, the damage of AChR-associated synaptic proteins aggravates the pathologic process and delays the repair process. Therefore, ideally, the goal of therapy should be to improve the potential restoration process of NMJ as well as discontinue the abnormal autoimmune process.
참고문헌 (20)
- Meriggioli MN, "Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity", Lancet Neurol, 8, pp.475~490 (2009)
- Hoch W, "Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies", Nat Med, 7, pp.365~368 (2001)
- Gomez AM, "Antibody effector mechanisms in myasthenia gravis-Pathogenesis at the neuromuscular junction", Autoimmunity, 43, pp.353~370 (2010)
- Engel AG, "The neuromuscular junction, In Myology", pp.325~372 (2004), McGraw-Hill
- Martinez-Martinez P, "Overexpression of rapsyn in rat muscle increases acetylcholine receptor levels in chronic experimental autoimmune myasthenia gravis", Am J Pathol, 170, pp.644~657 (2007)
- Ngo ST, "Neuregulin-1 potentiates agrin-induced acetylcholine receptor clustering through muscle-specific kinase phosphorylation", J Cell Sci, 125, pp.1531~1543 (2012)
- Li Q, "Synergistic effects of neuregulin and agrin on muscle acetylcholine receptor expression", Mol Cell Neurosci, 26, pp.558~569 (2004)
- Dumitru D, "Neuromuscular junction disorders, In Electrodiagnositc medicine", pp.1127~1228 (2002), Hanley & Belfus
- Gautam M, "Failure of postsynaptic specialization to develop at neuromuscular junctions of rapsyn-deficient mice", Nature, 377, pp.232~236 (1995)
- Ohno K, "Rapsyn mutations in humans cause endplate acetylcholine-receptor deficiency and myasthenic syndrome", Am J Hum Genet, 70, pp.875~885 (2002)
- Deconinck AE, "Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice", J Cell Biol, 136, pp.883~894 (1997)
- Zhang B, "LRP4 serves as a coreceptor of agrin", Neuron, 60, pp.285~297 (2008)
- Kim N, "Lrp4 is a receptor for agrin and forms a complex with MuSK", Cell, 135, pp.334~342 (2008)
- Weatherbee SD, "LDLreceptor- related protein 4 is crucial for formation of the neuromuscular junction", Development, 133, pp.4993~5000 (2006)
- Zhang B, "Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis", Arch Neurol, 69, pp.445~451 (2012)
- Yumoto N, "Lrp4 is a retrograde signal for presynaptic differentiation at neuromuscular synapses", Nature, 489, pp.438~442 (2012)
- 권기한, "MuSK항체 양성 중증근무력증", 대한근전도 전기진단의학회지, 14(1), pp.10~16 (2012), 대한근전도전기진단의학회
- Yamanashi Y, "Dok-7", MuSK signaling and a congenital myasthenic syndrome, 2008, pp.27, 25~29
- Okada K, "The muscle protein Dok-7 is essential for neuromuscular synaptogenesis", Science, 312, pp.1802~1805 (2006)
- Linnoila J, "A mammalian homolog of Drosophila tumorous imaginal discs, Tid1, mediates agrin signaling at the neuromuscular junction", Neuron, 60, pp.625~641 (2008)