C9

GWB-1F0814

735

002-94. 8. 8

This product recognises complement component 9 (C9) a 71kDa member of the complement C6/C7/C8/C9 family present in the blood serum and synthesised by the liver and monocytes. C9 is the last component to be added during the formation of the membrane attack complex (MAC) binding the membrane associated C5b-8 complex and resulting in the circular polymerisation of 12-18 C9 molecules. This forms the hydrophilic transmembrane channel which causes cell lysis. Deficiency of C9 is associated with recurring infections by Neisseria meningitides. This product does not recognise MAC.

Borate buffered saline pH8. 2 - 8. 4

C9 is the final component of the complement system to be added in the assembly of the membrane attack complex. It is able to enter lipid bilayers forming transmembrane channels.

Defects in C9 are a cause of component C9 deficiency (C9D) [MIM:120940]. Patients with C9D suffer from recurrent bacterial infections predominantly from Neisseria meningitidis.

Contains 1 EGF-like domain.

Contains 1 MACPF domain.