UP TO 88%
AChR autoantibodies

ARE DETECTED IN THE MAJORITY OF PATIENTS WITH gMG18-20

Autoantibody binding and resulting complement activation interrupt normal synaptic function needed to signal muscle contraction, resulting in the chronic muscle weakness that defines gMG.14-17

COMPLEMENT LEADS TO PROGRESSIVE AChR LOSS AND MEMBRANE DESTRUCTION17,21

Image
ACh, AChR
Image
ACh, AChR
  • In healthy individuals, ACh binding to densely packed AChRs initiates the signal for muscle contraction at the NMJ.17
  • The highly folded junctional membrane structure helps amplify the signal.17
 
ACh
ACh
Basement membrane
Basement Membrane
AChR
AChR
Image
AChR-Ab
Image
AChR-Ab
  • In AChR-Ab+ gMG, autoantibody binding to AChR recruits complement, resulting in formation of the MAC.17,21
  • MAC inserts into the membrane, causing focal lysis and triggering shedding of membrane fragments.17,21
 
C1q
AChR-Ab
AChR-Ab
MAC
MAC
Image
AChRs
  • AChRs are shed along with membrane fragments, reducing their number and activity.21
  • Membrane loss flattens junctional fold structure, further disrupting neuromuscular signal transmission.22