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The proximal complement pathway

Triggered via 3 different pathways: lectin, classical, and


alternative
Generation of C3 convertase complexes
facilitate the cleavage of C3, yielding C3a and C3b

PNH
C3
Convertase
(-) CD55

The terminal complement pathway


Initiated when C5b complexes with other factors, forming C5
convertases, which then cleave C5, yielding C5a and C5b

(-)
CD59

Theory # 3
Mutation in PIG a gene absence of GPI anchor
cannot attach CD157 and GPI-80 (fxn: mediating
granulocyte adhesiveness and chemotaxis) defective
trans-endothelial PMN migration and reduced
respiratory burst / apoptosis dysregulation in
neutrophil turnover Neutrophil mediated
inflammation fxn as Proinflammatory ALI / ARDS

Paroxysmal Nocturnal
Hemoglobinuria (PNH)
an acquired clonal non-malignant hematopoietic stem
cell disorder
Somatic mutation of the x-linked PIG-A gene prevents
the assembly of the GPI-anchor
Hemolyti
c
anemia
Bone
marrow
failure

PIG-A gene (phosphatidylinositol glycan complementation class A)


GPI (Glycosylphosphatidylinositol)

Thrombo
embolis
m

Clinical Triad of PNH

Proteins Deficient from PNH Blood Cells


CD55
CD58
CD59
PrPC
AChE
JMH Ag
Dombroch
HG Ag
CD55
CD58
CD59
CD109
PrPC
GP500
Gova/b

B cells

RBC

Haematopoietic
Stem Cell

CD24 CD55
CD58 CD59
CD48 PrPC CD73
CD108

T cells

CD59, CD90, CD109

Platelets

CD55 CD58*
CD59 CD48
CD52 CD87
CD108
PrPc
ADP-RT
CD73
CD90 CD109
CD16*

NK cells
CD55 CD58*
CD59 CD14
CD16 CD24
CD48 CD66b
CD66c
CD87
CD109
PrPc
LAPNB1
CD157
p50-80
GPI-80
ADP-RT
NA1/NA2

Monocytes
PMN

CD14
CD59
CD87
Group 8

CD55
CD48
CD109
PrPC
CD16

CD58*
CD52
CD157
GPI-80

CD55
CD58
CD59
CD48
CD52
PrPc
CD16

QuickTime and a
GIF decompressor
are needed to see this picture.

PNH (-)CD 157, (-)GPI80