Complement associated pathogenic mechanisms in myasthenia gravis

Erdem Tüzün, Premkumar Christadoss

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

The complement system is profoundly involved in the pathogenesis of acetylcholine receptor (AChR) antibody (Ab) related myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG). The most characteristic finding of muscle pathology in both MG and EAMG is the abundance of IgG and complement deposits at the nerve-muscle junction (NMJ), suggesting that AChR-Ab induces muscle weakness by complement pathway activation and consequent membrane attack complex (MAC) formation. This assumption has been supported with EAMG resistance of complement factor C3 knockout (KO), C4 KO and C5 deficient mice and amelioration of EAMG symptoms following treatment with complement inhibitors such as cobra venom factor, soluble complement receptor 1, anti-C1q, anti-C5 and anti-C6 Abs. Moreover, the complement inhibitor decay accelerating factor (DAF) KO mice exhibit increased susceptibility to EAMG. These findings have brought forward improvisation of novel therapy methods based on inhibition of classical and common complement pathways in MG treatment.

Original languageEnglish (US)
Pages (from-to)904-911
Number of pages8
JournalAutoimmunity Reviews
Volume12
Issue number9
DOIs
StatePublished - Jul 2013

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Autoimmune Experimental Myasthenia Gravis
Myasthenia Gravis
Complement Inactivating Agents
Cholinergic Receptors
CD55 Antigens
Complement C1
Classical Complement Pathway
Complement Membrane Attack Complex
Complement Receptors
Muscles
Complement C3
Antibodies
R Factors
Complement Activation
Muscle Weakness
Knockout Mice
Animal Models
Immunoglobulin G
Pathology

Keywords

  • Autoimmunity
  • Classical pathway
  • Complement
  • Experimental autoimmune myasthenia gravis
  • Myasthenia gravis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Complement associated pathogenic mechanisms in myasthenia gravis. / Tüzün, Erdem; Christadoss, Premkumar.

In: Autoimmunity Reviews, Vol. 12, No. 9, 07.2013, p. 904-911.

Research output: Contribution to journalArticle

Tüzün, Erdem ; Christadoss, Premkumar. / Complement associated pathogenic mechanisms in myasthenia gravis. In: Autoimmunity Reviews. 2013 ; Vol. 12, No. 9. pp. 904-911.
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