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Autoantibodies in gMG disrupt neuromuscular communication, resulting in impaired synaptic signaling and subsequent weakness and fatigability.2

Upstream production of acetylcholine receptor positive (AChR+) and muscle-specific kinase positive (MuSK+) autoantibodies initiates key disease processes at the neuromuscular junction.1,*

Pathogenic B cell contained icon

Pathogenic B cells are the primary source of autoantibody production.1,3,4

Plasmacell icon

CD19+ plasmablasts and plasma cells produce pathogenic autoantibodies to MuSK and AChR.2,3

autoantibodies icon

Following production by B cells, AChR and MuSK autoantibodies play a primary role in gMG pathophysiology and are responsible for key disease processes.5

AChR autoantibodies icon

AChR+ gMG is mediated by AChR autoantibodies via 3 mechanisms:5

  • Blocking
  • Cross-modulation (cross-linking)
  • Complement-mediated damage
MuSK autoantibodies icon

MuSK+ gMG is mediated by MuSK+ autoantibodies, which disrupt the AChR clustering process.​5

  • Distinct Markers in the B-Cell Lineage: What’s the Difference?

    As B cells develop, they express different markers, like CD19 and CD20. Understanding where these appear in the lineage may help clarify their role in autoantibody production.6

    Explore B-cell lineage in gMG

*In LRP4+ gMG, autoantibodies target the LRP4-signaling cascade and impair AChR clustering.7
LRP4+, low-density lipoprotein receptor-related protein 4 antibody positive.

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Review the 3 serotypes of gMG
  • References

    1. Yi JS, Guptill JT, Stathopoulos P, Nowak RJ, O'Connor KC. Muscle Nerve. 2018;56(2):172-184.

    2. Huda R. Front Immunol. 2020;11:510568.

    3. Fichtner ML, Jiang R, Bourke A, Nowak RJ, O’Connor KC. Front Immunol. 2020;11:776.

    4. Sun B, Ramberger M, O’Connor KC, Bashford-Rogers RJM, Irani SR. Nat Rev Neurol. 2020;16:481-492.

    5. Masi G, O’Connor KC. Curr Opin Neurol. 2022;35:586-596.

    6. Forsthuber TG, Cimbora DM, Ratchford JN, et al. Ther Adv Neurol Disord. 2018;11:1-13.

    7. Dresser L, Wlodarski R, Rezania K, Soliven B. J Clin Med. 2021;10:2235.