argenx SE Investor Day Presentation Deck
Anti-GM1 Antibodies Activate Classical Complement Pathway
1
IgM autoantibodies
bind to GM1 located on
motor neurons and
Schwann cells
3
Complement activity
correlates with
disease severity
argenx
IgM
muscle
CP activity (%)
Schwann cell
110
100
90
80
axon
motor endplate
Minor
myelin
node of Ranvier
+
GM1
Vlam et al, Neurol Neuroimmunol Neuroinflamm. 2015
Yuki et al, J Peripheral Nervous System. 2011
cell body
Į
Mild Moderate Severe
Muscle weakness
2
IgM autoantibodies are potent activators
of the complement cascade
IgM titer is correlated with classical
pathway complement deposition
4
SOC IVlg reduces
C3b deposition
A
C3b deposition (OD)
Complement depositon (OD)
تن
2
-
0
0
2.5
XO
O
-------..
5
-- C1q
-* C4b
→ C3b
2
Anti-GM1 antibody titer (OD)
OHSA
10
Concentration (mg/ml)
3
IVIG
IgM autoantibodies cannot be addressed by efgartigimod
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