# keratin – epithelium
# vimentin – mesenchyme
# desmin – muscle
# neurofilament – neuron
# GFAP – glia
Daily Archives: 09/15/2012
Paroxysmal nocturnal hematuria
# PNH results from somatic mutation in PIGA gene which leads to the shortage of GPI
# GPI anchors protective proteins (CD55 and CD59) to the surface of RBC
– CD55 blocks C3bBb (C3 convertase from alternative pathway)
– CD59 blocks the formation of MAC through binding to C8 and (C9)n
# Eculizumab binds specifically to C5 and blocks the action of C5 convertase
– RBC is still subject to C3b opsonization