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KCI 후보

중증 근무력증에서 일어나는 신경근접합부의 면역병리학적 변화

Immunopathologic Change of Neuromuscular Junction in Myasthenia Gravis

대한근전도 전기진단의학회지
약어 : J Electrodiagn Neuromuscul Dis
2013 vol.15, no.1, pp.6 - 10
DOI : 10.18214/jkaem.2013.15.1.6
발행기관 : 대한근전도전기진단의학회
연구분야 : 재활의학
Copyright © 대한근전도전기진단의학회
197 회 열람

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.

Myasthenia gravis, Neuromuscular junction, Autoimmune diseases, Autoantibodies, Proteins

  • 1. [학술지] Meriggioli MN / 2009 / Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity / Lancet Neurol / 8 : 475 ~
  • 2. [학술지] Hoch W / 2001 / Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies / Nat Med / 7 : 365 ~
  • 3. [학술지] Gomez AM / 2010 / Antibody effector mechanisms in myasthenia gravis-Pathogenesis at the neuromuscular junction / Autoimmunity / 43 : 353 ~
  • 4. [단행본] Engel AG / 2004 / The neuromuscular junction, In Myology / McGraw-Hill : 325 ~
  • 5. [학술지] Martinez-Martinez P / 2007 / Overexpression of rapsyn in rat muscle increases acetylcholine receptor levels in chronic experimental autoimmune myasthenia gravis / Am J Pathol / 170 : 644 ~
  • 6. [학술지] Ngo ST / 2012 / Neuregulin-1 potentiates agrin-induced acetylcholine receptor clustering through muscle-specific kinase phosphorylation / J Cell Sci / 125 : 1531 ~
  • 7. [학술지] Li Q / 2004 / Synergistic effects of neuregulin and agrin on muscle acetylcholine receptor expression / Mol Cell Neurosci / 26 : 558 ~
  • 8. [단행본] Dumitru D / 2002 / Neuromuscular junction disorders, In Electrodiagnositc medicine / Hanley & Belfus : 1127 ~
  • 9. [학술지] Gautam M / 1995 / Failure of postsynaptic specialization to develop at neuromuscular junctions of rapsyn-deficient mice / Nature / 377 : 232 ~
  • 10. [학술지] Ohno K / 2002 / Rapsyn mutations in humans cause endplate acetylcholine-receptor deficiency and myasthenic syndrome / Am J Hum Genet / 70 : 875 ~
  • 11. [학술지] Deconinck AE / 1997 / Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice / J Cell Biol / 136 : 883 ~
  • 12. [학술지] Zhang B / 2008 / LRP4 serves as a coreceptor of agrin / Neuron / 60 : 285 ~
  • 13. [학술지] Kim N / 2008 / Lrp4 is a receptor for agrin and forms a complex with MuSK / Cell / 135 : 334 ~
  • 14. [학술지] Weatherbee SD / 2006 / LDLreceptor- related protein 4 is crucial for formation of the neuromuscular junction / Development / 133 : 4993 ~
  • 15. [학술지] Zhang B / 2012 / Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis / Arch Neurol / 69 : 445 ~
  • 16. [학술지] Yumoto N / 2012 / Lrp4 is a retrograde signal for presynaptic differentiation at neuromuscular synapses / Nature / 489 : 438 ~
  • 17. [학술지] 권기한 / 2012 / MuSK항체 양성 중증근무력증 / 대한근전도 전기진단의학회지 / 14 (1) : 10 ~ 1 kci
  • 18. [학술지] Yamanashi Y / 2008 / Dok-7/MuSK signaling and a congenital myasthenic syndrome / Acta Myol / 27 : 25 ~
  • 19. [학술지] Okada K / 2006 / The muscle protein Dok-7 is essential for neuromuscular synaptogenesis / Science / 312 : 1802 ~
  • 20. [학술지] Linnoila J / 2008 / A mammalian homolog of Drosophila tumorous imaginal discs, Tid1, mediates agrin signaling at the neuromuscular junction / Neuron / 60 : 625 ~