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 language | English (US) |
|---|---|
| Pages (from-to) | 904-911 |
| Number of pages | 8 |
| Journal | Autoimmunity Reviews |
| Volume | 12 |
| Issue number | 9 |
| DOIs | |
| State | Published - Jul 2013 |
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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 journal › Article
}
TY - JOUR
T1 - Complement associated pathogenic mechanisms in myasthenia gravis
AU - Tüzün, Erdem
AU - Christadoss, Premkumar
PY - 2013/7
Y1 - 2013/7
N2 - 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.
AB - 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.
KW - Autoimmunity
KW - Classical pathway
KW - Complement
KW - Experimental autoimmune myasthenia gravis
KW - Myasthenia gravis
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UR - http://www.scopus.com/inward/citedby.url?scp=84879177463&partnerID=8YFLogxK
U2 - 10.1016/j.autrev.2013.03.003
DO - 10.1016/j.autrev.2013.03.003
M3 - Article
VL - 12
SP - 904
EP - 911
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
SN - 1568-9972
IS - 9
ER -